Rheumatological emergencies
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Rheumatological emergencies
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Rheumatological emergencies: topic map
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Acute crystal arthritis
- Septic arthritis must be excluded before assuming gout or CPPD.
- Arthrocentesis, microscopy, Gram stain and culture are central.
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Septic arthritis
- A rapidly destructive joint infection requiring urgent aspiration, antibiotics and often surgical drainage.
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Acute spinal emergencies
- Atlanto-axial subluxation and cervical myelopathy in rheumatoid arthritis.
- Spinal epidural abscess can mimic an inflammatory flare.
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Systemic vasculitis emergencies
- Pulmonary-renal syndrome, diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis.
- Mesenteric ischemia, mononeuritis multiplex, and critical limb ischemia.
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Giant cell arteritis
- Threatened or established visual loss requires immediate glucocorticoids, without awaiting biopsy or imaging.
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Catastrophic antiphospholipid syndrome
- Acute thromboses in multiple organs, often with thrombocytopenia and microangiopathy.
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Systemic lupus erythematosus emergencies
- Lupus nephritis with rapidly deteriorating renal function, neuropsychiatric lupus, severe cytopenias, myocarditis, pulmonary hemorrhage.
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Scleroderma renal crisis
- Abrupt hypertension and acute kidney injury, managed urgently with an ACE inhibitor.
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Inflammatory myopathy emergencies
- Respiratory-muscle weakness, severe dysphagia with aspiration, and rhabdomyolysis.
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Macrophage activation syndrome / secondary HLH
- Persistent high fever, cytopenias, liver dysfunction, coagulopathy and markedly high ferritin, often complicating systemic JIA or adult-onset Still disease.
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Drug and treatment-related emergencies
- Serious infection in immunosuppressed patients, neutropenic sepsis, infusion reactions, and medication toxicities.
High-level approach
A rheumatological emergency is usually one of three patterns: infection mistaken for inflammation, organ-threatening immune disease, or thrombotic/vascular occlusion. Initial management prioritizes ABCDE assessment, sepsis evaluation, urgent specialist involvement, and targeted tests such as joint aspiration, urinalysis with renal function, full blood count, coagulation tests, inflammatory markers, cultures, chest imaging, and autoimmune studies where appropriate.
Do not delay time-critical treatment when there is suspected bacterial sepsis, threatened vision in giant cell arteritis, pulmonary hemorrhage, rapidly progressive renal failure, hypertensive renal crisis, or multi-organ thrombosis.
Which topic should I cover in detail?
- Exam-oriented complete outline
- Emergency recognition and initial management
- Vasculitis and pulmonary-renal syndromes
- SLE, APS, scleroderma and MAS
Exam oriented complete outline
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rheumatological emergencies septic arthritis giant cell arteritis scleroderma renal crisis emergency management guideline
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2025 guideline giant cell arteritis immediate glucocorticoids visual symptoms septic arthritis native joint guideline
Rheumatological Emergencies: Exam-Oriented Complete Outline
Definition
Rheumatological emergencies are conditions related to inflammatory, autoimmune, crystal, thrombotic, infective, or treatment-related rheumatic disease that can cause rapid organ loss, irreversible disability, or death without urgent recognition and treatment.
Universal approach in the emergency setting
1. Recognize red flags
- Fever, hypotension, tachycardia, altered sensorium
- Acutely red, hot, swollen joint
- Visual symptoms or new temporal headache
- Hemoptysis, hypoxia, falling hemoglobin
- Acute kidney injury, hematuria, proteinuria, hypertension
- Purpura, digital ischemia, mononeuritis multiplex
- Cytopenias, hepatitis, coagulopathy, hyperferritinemia
- Severe muscle weakness, dysphagia, respiratory weakness
- Multi-organ thrombosis
2. Initial investigations
- CBC with differential and platelet count
- ESR, CRP, renal and liver function tests
- Urinalysis and urine microscopy
- Blood cultures before antibiotics if sepsis is possible
- Coagulation profile, LDH, haptoglobin, blood film where thrombotic microangiopathy is possible
- Creatine kinase in suspected myositis/rhabdomyolysis
- Ferritin, triglycerides, fibrinogen in suspected MAS/HLH
- Chest radiograph or CT chest for pulmonary involvement
- Autoimmune tests guided by syndrome: ANA, dsDNA, complement, ANCA, anti-GBM, antiphospholipid antibodies
- Urgent specialist input: rheumatology plus the affected organ specialty, ICU, orthopedics, ophthalmology, nephrology, hematology, or respiratory medicine.
3. Core rule
Do not attribute acute deterioration to a rheumatologic flare until infection, thrombosis, drug toxicity, and malignancy have been considered.
1. Septic arthritis
Why it is an emergency
Rapid cartilage destruction, permanent loss of joint function, bacteremia, and death can occur. Fever may be absent, especially in older adults and immunosuppressed patients.
The SANJO guideline states that no clinical parameter alone can confidently rule septic arthritis in or out, and a painful, inflamed joint warrants high suspicion. SANJO guideline
Risk factors
- Rheumatoid arthritis or other chronic arthropathy
- Prosthetic joint
- Immunosuppression, including glucocorticoids and biologics
- Diabetes, chronic kidney disease, cirrhosis
- IV drug use
- Recent joint injection, surgery, skin infection, or bacteremia
Clinical features
- Acute monoarthritis: severe pain, swelling, warmth, restricted active and passive movement
- Knee most common, then hip, shoulder, wrist, ankle
- Fever or systemic toxicity may occur
- Polyarticular disease can occur in rheumatoid arthritis and disseminated gonococcal infection.
Diagnosis
- Urgent arthrocentesis before antibiotics, if this does not delay resuscitation in septic shock.
- Send synovial fluid for:
- Cell count and differential
- Gram stain
- Culture and sensitivity
- Crystal analysis
- Obtain blood cultures.
- A negative Gram stain or crystals in aspirate do not exclude concurrent infection.
Management
- ABCDE and sepsis management.
- Prompt IV empirical antibiotics after cultures.
- Urgent orthopedic consultation for repeated aspiration, arthroscopic washout, or open drainage.
- Treat as septic arthritis until excluded.
Exam pearl: A hot swollen joint in an immunosuppressed patient is septic arthritis until proven otherwise.
2. Giant cell arteritis: visual emergency
Why it is an emergency
GCA can cause sudden irreversible visual loss due to ischemic optic neuropathy. It needs treatment immediately if strongly suspected, without waiting for temporal artery biopsy or imaging.
The American College of Rheumatology notes that treatment should begin immediately to prevent visual loss. ACR GCA guidance
Suspect GCA in a patient aged over 50 with
- New-onset temporal or generalized headache
- Scalp tenderness
- Jaw or tongue claudication
- Visual blurring, diplopia, amaurosis fugax, or visual loss
- Polymyalgia rheumatica symptoms
- Constitutional symptoms
- Raised ESR and CRP, though normal markers do not completely exclude it.
Investigations
- ESR, CRP, CBC, liver function tests
- Temporal artery ultrasound where expertise is available
- Temporal artery biopsy, ideally soon after treatment begins
- Urgent ophthalmology review for visual symptoms.
Management
- No visual ischemia: high-dose oral glucocorticoid therapy, commonly prednisolone 40-60 mg/day.
- Threatened or established visual loss: urgent IV methylprednisolone is generally preferred, followed by high-dose oral glucocorticoids.
- Do not delay steroids for biopsy.
- Long-term care includes gradual tapering, relapse surveillance, steroid-toxicity prevention, and consideration of steroid-sparing treatment such as tocilizumab under specialist care.
Exam pearl: Jaw claudication is highly suggestive; visual symptoms require immediate glucocorticoids.
3. Pulmonary-renal syndrome and diffuse alveolar hemorrhage
Definition
Pulmonary-renal syndrome is diffuse alveolar hemorrhage plus glomerulonephritis. It is most often caused by:
- ANCA-associated vasculitis: GPA or MPA
- Anti-GBM disease
- SLE
- Less often, IgA vasculitis or cryoglobulinemic vasculitis
Presentation
- Dyspnea, cough, hypoxia
- Hemoptysis may be absent
- Falling hemoglobin and diffuse pulmonary infiltrates
- Acute kidney injury
- Hematuria, proteinuria, dysmorphic red cells, red-cell casts
- Hypertension and systemic vasculitic features.
Diffuse alveolar hemorrhage often presents with dyspnea, hemoptysis, alveolar opacity, and anemia, but hemoptysis may be absent. Pulmonary-renal syndrome requires urgent evaluation. Comprehensive Clinical Nephrology, 7th Edition, p. 1381.
Immediate management
- Admit to HDU/ICU if hypoxic or rapidly deteriorating.
- Oxygen and ventilatory support where needed.
- Urgent nephrology and rheumatology input.
- Urinalysis, renal function, ANCA, anti-GBM, complements, ANA/dsDNA.
- Consider bronchoscopy with bronchoalveolar lavage if diagnosis is uncertain and patient is stable enough.
- Renal biopsy is often needed, but do not delay treatment in life-threatening disease.
- High-dose glucocorticoids plus disease-specific immunosuppression, commonly rituximab or cyclophosphamide for severe ANCA vasculitis.
- Anti-GBM disease usually requires plasma exchange plus immunosuppression.
- Exclude infection before escalating immunosuppression.
Exam pearl: Hemoptysis + acute kidney injury + active urinary sediment = pulmonary-renal syndrome until proved otherwise.
4. Systemic vasculitis with organ-threatening disease
Red-flag manifestations
- Rapidly progressive glomerulonephritis
- Alveolar hemorrhage
- Mononeuritis multiplex or acute foot/wrist drop
- Mesenteric ischemia or gastrointestinal perforation
- Digital ischemia or gangrene
- CNS involvement: seizures, stroke, encephalopathy
- Scleritis or retinal vasculitis
- Myocarditis or coronary vasculitis
Key clinical patterns
| Condition | High-yield clues | Emergency risk |
|---|---|---|
| GPA | ENT disease, pulmonary nodules/hemorrhage, glomerulonephritis, PR3-ANCA | Pulmonary-renal syndrome |
| MPA | Pulmonary capillaritis, glomerulonephritis, MPO-ANCA | DAH, rapidly progressive GN |
| PAN | Hypertension, abdominal pain, neuropathy, livedo | Mesenteric or renal ischemia |
| Cryoglobulinemic vasculitis | Purpura, neuropathy, low C4, hepatitis C association | Renal and neurologic involvement |
| Behçet disease | Oral/genital ulcers, uveitis, thrombosis | Cerebral venous thrombosis, arterial aneurysm |
| Takayasu arteritis | Pulse asymmetry, bruits, hypertension | Cerebrovascular or cardiac ischemia |
Principles of management
- Stabilize affected organ system.
- Obtain cultures and infection screen.
- Give high-dose glucocorticoids when life or organ is threatened.
- Add induction immunosuppression under specialist supervision.
- Tissue diagnosis, often kidney, skin, nerve, lung, or temporal artery biopsy, should be pursued when safe but must not delay lifesaving therapy.
5. Scleroderma renal crisis
Definition
An acute, life-threatening complication of systemic sclerosis characterized by:
- Abrupt severe hypertension, sometimes normotensive
- Acute kidney injury
- Thrombotic microangiopathy and microangiopathic hemolytic anemia
- Hypertensive encephalopathy, heart failure, or retinopathy in severe cases
Scleroderma renal crisis presents with accelerated hypertension, acute kidney injury, and progressive renal failure. Harrison's Principles of Internal Medicine, 22nd Edition, p. 2651.
Risk factors
- Early diffuse cutaneous systemic sclerosis
- Rapid skin thickening
- Anti-RNA polymerase III antibodies
- Recent high-dose glucocorticoid exposure
- Tendon friction rubs
Investigations
- BP monitoring, renal function, urinalysis
- CBC, blood film, LDH, bilirubin, haptoglobin
- ECG, echocardiography if heart failure suspected
- Fundoscopy where available.
Management
- Start ACE inhibitor immediately, classically captopril because rapid titration is possible.
- Aggressively control BP with close monitoring.
- Do not stop ACE inhibitor solely because creatinine rises initially.
- Dialysis may be temporarily required.
- Avoid high-dose glucocorticoids unless another compelling indication exists.
Exam pearl: In systemic sclerosis with sudden hypertension and AKI, start an ACE inhibitor urgently.
6. Catastrophic antiphospholipid syndrome
Definition
Catastrophic APS is an accelerated thrombotic storm causing small-vessel thrombosis and rapidly progressive failure of three or more organ systems, typically developing within a week.
Triggers
- Infection
- Surgery or trauma
- Anticoagulation withdrawal
- Pregnancy/postpartum state
- SLE flare
- Malignancy
Clinical features
- Renal failure
- Encephalopathy, stroke, seizures
- Respiratory failure/ARDS
- Myocardial ischemia
- Abdominal pain from mesenteric thrombosis
- Skin necrosis, livedo
- Thrombocytopenia and hemolytic anemia
Investigations
- CBC and blood film
- PT, aPTT, fibrinogen, D-dimer
- Renal and liver function
- Lupus anticoagulant, anticardiolipin, anti-beta-2 glycoprotein I antibodies
- Imaging to identify thrombosis
- Evaluate mimics: DIC, TTP, HUS, HIT, sepsis.
Management
- ICU-level multidisciplinary care.
- Therapeutic anticoagulation, usually IV unfractionated heparin.
- High-dose glucocorticoids.
- Plasma exchange and/or IV immunoglobulin for severe disease.
- Treat trigger, particularly infection.
- Consider cyclophosphamide if CAPS occurs with active SLE.
- Refractory cases require specialist-directed therapies.
Exam pearl: Multiorgan thrombosis over days in a patient with APS is CAPS, not simply recurrent venous thromboembolism.
7. SLE emergencies
A. Lupus nephritis with rapidly progressive renal failure
Features
- Rising creatinine
- Proteinuria, hematuria, red-cell casts
- Hypertension, edema
- Low complement, rising anti-dsDNA may support active lupus.
Management
- Urgent nephrology-rheumatology referral.
- Renal biopsy where possible.
- High-dose glucocorticoids plus induction immunosuppression according to biopsy class and severity.
- Exclude infection, drug toxicity, thrombotic microangiopathy, and APS nephropathy.
B. Diffuse alveolar hemorrhage
- Dyspnea, falling hemoglobin, hypoxia, pulmonary infiltrates.
- Manage in ICU with high-dose glucocorticoids and specialist immunosuppression after infection evaluation.
C. Neuropsychiatric lupus
- Seizures, psychosis, acute confusional state, stroke, myelitis.
- Exclude infection, metabolic disease, drug effects, thrombosis, and posterior reversible encephalopathy syndrome.
- MRI brain/spine, CSF when appropriate, and APS testing.
D. Severe autoimmune cytopenias
- Immune thrombocytopenia with bleeding
- Autoimmune hemolytic anemia
- Neutropenia with sepsis risk
- Treat with glucocorticoids, IVIG in selected urgent cases, transfusion support as clinically indicated.
8. Macrophage activation syndrome and secondary HLH
Definition
MAS is a form of secondary hemophagocytic lymphohistiocytosis caused by uncontrolled immune activation. It is classically associated with systemic JIA, adult-onset Still disease, and SLE.
Clinical features
- Persistent high fever
- Hepatosplenomegaly
- Cytopenias
- Hepatitis, rising transaminases
- Coagulopathy, low fibrinogen
- Hypertriglyceridemia
- Very high ferritin
- Neurologic involvement
- Multiorgan failure
In systemic JIA, ferritin greater than 684 micrograms/L plus any two of thrombocytopenia, raised AST, hypertriglyceridemia, or low fibrinogen supports classification of MAS. Rheumatology, 2-Volume Set, p. 6089.
Important distinction
An apparent fall in ESR in a highly inflamed patient can be ominous because fibrinogen is consumed.
Management
- ICU assessment if unstable.
- Screen aggressively for infection and malignancy.
- High-dose glucocorticoids.
- Specialist-directed anakinra, cyclosporine, IVIG, or other immunomodulation depending on cause and severity.
- Support organ failure and treat triggers.
Exam pearl: Fever + falling cell counts + high ferritin + low fibrinogen in Still disease/systemic JIA strongly suggests MAS.
9. Inflammatory myopathy emergencies
Emergency manifestations
- Respiratory muscle weakness causing type 2 respiratory failure
- Severe bulbar weakness and dysphagia causing aspiration
- Rapidly progressive weakness
- Rhabdomyolysis with acute kidney injury
- Myocarditis or arrhythmia in selected inflammatory myopathies
Assessment
- Forced vital capacity and negative inspiratory force
- Arterial blood gas if respiratory compromise
- Swallow assessment
- CK, electrolytes, renal function, ECG, troponin when cardiac involvement is possible
- Assess for infection and drug-induced myopathy.
Management
- ICU or respiratory support when required.
- Aspiration precautions and enteral feeding plan.
- High-dose glucocorticoids plus early steroid-sparing immunosuppression for severe confirmed inflammatory disease.
- IVIG is particularly useful in severe dysphagia or selected refractory cases.
- Manage rhabdomyolysis with fluids and electrolyte correction.
10. Cervical spine instability in rheumatoid arthritis
Mechanism
Chronic RA may cause atlanto-axial subluxation, basilar invagination, or subaxial cervical instability.
Red flags
- Neck pain or occipital headache
- Myelopathy: gait disturbance, hyperreflexia, weakness
- Paresthesia, sphincter symptoms
- Symptoms after minor trauma
- Difficulty positioning for intubation.
Management
- Immobilize the neck if instability is suspected.
- Urgent CT/MRI and spinal surgical review.
- Inform anesthesiology before airway manipulation.
Exam pearl: Never force neck extension for intubation in RA with suspected cervical spine involvement.
11. Acute crystal arthritis: the septic arthritis trap
Gout and CPPD may cause severe inflammatory monoarthritis but are usually not life-threatening. The emergency is failure to recognize coexistent septic arthritis.
Acute gout
- Abrupt, severe pain, classically first MTP but can involve any joint.
- Needle-shaped, negatively birefringent monosodium urate crystals.
Acute CPPD
- Commonly knee or wrist.
- Rhomboid, weakly positively birefringent crystals.
- Consider metabolic associations in younger patients.
Principle
Perform joint aspiration when infection is plausible. Crystals do not exclude infection.
12. Drug-related emergencies in rheumatology
Serious infection
Patients receiving glucocorticoids, conventional DMARDs, biologics, JAK inhibitors, or cyclophosphamide may have muted fever and inflammatory-marker responses.
- Consider bacterial sepsis, opportunistic infection, tuberculosis, Pneumocystis pneumonia, herpes zoster, and invasive fungal infection according to therapy and risk factors.
- Withhold immunosuppressive therapy in serious infection unless a specialist directs otherwise.
Methotrexate toxicity
- Mucositis, pancytopenia, renal failure, transaminitis.
- Risk increased by renal impairment and interacting medications.
- Urgent blood counts, renal/liver tests, infection assessment, and specialist toxicology/hematology input.
Cyclophosphamide toxicity
- Myelosuppression, hemorrhagic cystitis, infection.
- Neutropenic fever is a medical emergency.
Short-answer exam framework
When asked to list rheumatological emergencies, write:
- Septic arthritis
- Giant cell arteritis with threatened visual loss
- Pulmonary-renal syndrome due to vasculitis, anti-GBM disease, or SLE
- Rapidly progressive glomerulonephritis
- Diffuse alveolar hemorrhage
- Scleroderma renal crisis
- Catastrophic antiphospholipid syndrome
- Macrophage activation syndrome/secondary HLH
- Severe SLE: nephritis, CNS lupus, cytopenias, myocarditis, pulmonary hemorrhage
- Severe systemic vasculitis with organ ischemia
- Inflammatory myopathy with respiratory or bulbar weakness
- RA-related atlanto-axial subluxation
- Serious infection and drug toxicity in immunosuppressed patients
One-line revision points
- Hot swollen joint: aspirate urgently and treat as septic until excluded.
- GCA plus visual symptom: give glucocorticoid immediately.
- Hemoptysis plus AKI: suspect pulmonary-renal syndrome.
- Scleroderma plus hypertension and AKI: start ACE inhibitor urgently.
- APS plus rapid multiorgan thrombosis: treat as CAPS with anticoagulation and immunomodulatory therapy.
- Still disease/systemic JIA plus cytopenias and high ferritin: suspect MAS.
- RA plus neurological signs or neck pain: consider cervical instability.
Find the latest guidelines for rheumatological emergencies and exam style SBA & viva question set
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2024 2025 2026 guideline rheumatology emergency septic arthritis giant cell arteritis ANCA vasculitis scleroderma renal crisis macrophage activation syndrome
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latest guideline scleroderma renal crisis CAPS macrophage activation syndrome HLH 2024 2025 EULAR consensus
Latest guideline map for rheumatological emergencies
There is no single guideline covering every rheumatological emergency. In exams and clinical practice, use the most recent disease-specific guideline alongside local sepsis, ICU, renal, and thrombosis protocols.
| Emergency | Most current high-value guideline | Exam-relevant message |
|---|---|---|
| Septic arthritis | GEIO/SEIMC/SEIP/SECOT adult and paediatric guideline, 2024 [PMID: 37919201]; also SANJO guideline, 2023 | Treat a painful, hot, swollen joint as septic until excluded. Aspirate promptly for microscopy, culture and crystals. Obtain blood cultures. Start IV antimicrobials after sampling, unless sepsis mandates immediate treatment. Arrange source control, commonly surgical washout or serial aspiration. |
| Giant cell arteritis with visual symptoms | ACR/Vasculitis Foundation guideline, 2021 [PMID: 34235884]; BSR guideline, 2020 [PMID: 31970405] | GCA is a vision-threatening emergency. Start glucocorticoids immediately when strongly suspected. Do not wait for temporal artery biopsy or imaging. Threatened visual loss generally warrants IV pulse glucocorticoids under urgent specialist care. |
| ANCA-associated vasculitis with pulmonary-renal syndrome or rapidly progressive GN | BSR recommendations, 2025 [PMID: 40499922]; KDIGO guideline, 2024 [PMID: 38388102] | Life- or organ-threatening AAV requires urgent glucocorticoid-based induction plus rituximab or cyclophosphamide, chosen according to clinical setting. Investigate urgently for ANCA, anti-GBM antibody, renal involvement, and infection. |
| Scleroderma renal crisis | BSR systemic sclerosis guideline, 2024 [PMID: 39255973] | Recognize abrupt hypertension with AKI and possible thrombotic microangiopathy. Start an ACE inhibitor immediately and titrate rapidly. Do not withhold ACE inhibition solely because creatinine initially rises. Monitor BP closely in patients exposed to glucocorticoids. |
| Severe SLE including nephritis, alveolar hemorrhage, neuropsychiatric SLE and cytopenias | ACR SLE guideline, 2025 [PMID: 41187097], published 2026; BSR SLE guideline, 2026 [PMID: 42336388] | First distinguish active lupus from infection, thrombotic APS, drug toxicity, and hypertensive emergency. Severe organ involvement requires multidisciplinary care and prompt immunosuppression tailored to organ disease. |
| Macrophage activation syndrome/secondary HLH | HiHASC adult HLH consensus guideline, 2024 [PMID: 38258680] | Think of MAS/HLH in persistent fever with cytopenias, hepatitis, coagulopathy, high ferritin and falling fibrinogen. Simultaneously seek infection, malignancy, and autoimmune triggers. Treat in collaboration with rheumatology, hematology, and ICU. |
| Catastrophic antiphospholipid syndrome | No newer universally adopted CAPS-specific guideline was identified. The commonly cited multidisciplinary framework remains the 2018 McMaster RARE-Best Practices guideline. | Suspect in rapidly developing thromboses affecting 3 or more organs. Emergency treatment is therapeutic anticoagulation, glucocorticoids, plasma exchange and/or IVIG, plus treatment of a trigger. Add cyclophosphamide when associated with active SLE. |
| Diffuse alveolar hemorrhage/pulmonary-renal syndrome | Use the AAV and KDIGO guidance above, with disease-specific anti-GBM or SLE management. | Hemoptysis may be absent. Dyspnea, falling hemoglobin, new diffuse infiltrates, AKI and active urine sediment require ICU-level evaluation. |
High-yield guideline updates
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AAV: The 2025 BSR guideline and 2024 KDIGO guideline are the current key references for severe renal or pulmonary vasculitis. Plasma exchange is not routine for all AAV, and its use should be individualized, especially in severe renal failure or diffuse alveolar hemorrhage.
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GCA: Immediate therapy matters more than diagnostic delay. Confirm with ultrasound, biopsy, or other vascular imaging as locally appropriate, but only after treatment has begun if suspicion is high.
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Systemic sclerosis: ACE inhibition remains the first-line emergency treatment for scleroderma renal crisis. Avoid unnecessary high-dose glucocorticoids because they increase risk.
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Septic arthritis: Crystal identification does not exclude joint infection. A negative Gram stain also does not exclude it.
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MAS/HLH: A decline in ESR during ongoing systemic inflammation may be a warning sign because fibrinogen is being consumed.
A current evidence caveat: broad, recent systematic reviews specifically titled “rheumatological emergencies” are scarce. Management is driven mainly by the disease-specific guidelines above rather than a single umbrella guideline.
SBA question set
SBA 1: Hot swollen knee
A 63-year-old woman with rheumatoid arthritis on prednisolone and a biologic agent presents with a painful swollen knee, temperature 37.8°C, and CRP 180 mg/L. Synovial fluid contains monosodium urate crystals. What is the best next step?
A. Diagnose gout and prescribe colchicine
B. Diagnose rheumatoid flare and increase prednisolone
C. Send synovial fluid for Gram stain and culture, obtain blood cultures, and manage as possible septic arthritis
D. Discharge with NSAIDs because crystals explain the symptoms
E. Start allopurinol immediately
B. Diagnose rheumatoid flare and increase prednisolone
C. Send synovial fluid for Gram stain and culture, obtain blood cultures, and manage as possible septic arthritis
D. Discharge with NSAIDs because crystals explain the symptoms
E. Start allopurinol immediately
Answer: C. Crystals do not exclude septic arthritis. Immunosuppression increases infection risk and may blunt fever.
SBA 2: GCA
A 76-year-old woman has a new temporal headache, jaw claudication, ESR 96 mm/hour, and brief episodes of painless monocular visual loss. What is the most appropriate immediate management?
A. Wait for temporal artery biopsy
B. Start oral methotrexate
C. Give immediate high-dose glucocorticoids and arrange urgent ophthalmology/rheumatology review
D. Arrange outpatient MRI brain
E. Give aspirin alone
B. Start oral methotrexate
C. Give immediate high-dose glucocorticoids and arrange urgent ophthalmology/rheumatology review
D. Arrange outpatient MRI brain
E. Give aspirin alone
Answer: C. This is threatened visual loss due to suspected GCA. Treatment must not wait for biopsy.
SBA 3: Pulmonary-renal syndrome
A 42-year-old man has worsening dyspnea, bilateral pulmonary infiltrates, hemoglobin falling from 11.5 to 8.2 g/dL, creatinine 3.0 mg/dL, and urine microscopy showing dysmorphic red cells and red-cell casts. Which diagnosis is most likely?
A. Cardiogenic pulmonary edema
B. Pulmonary embolism
C. Pulmonary-renal syndrome
D. Community-acquired pneumonia
E. Nephrotic syndrome
B. Pulmonary embolism
C. Pulmonary-renal syndrome
D. Community-acquired pneumonia
E. Nephrotic syndrome
Answer: C. Diffuse alveolar hemorrhage with glomerulonephritis is pulmonary-renal syndrome, most often due to AAV, anti-GBM disease, or SLE.
SBA 4: Investigation in pulmonary-renal syndrome
In the preceding patient, which test is particularly important because it may alter urgent management?
A. Rheumatoid factor
B. Anti-GBM antibody
C. Serum urate
D. HLA-B27
E. Anti-CCP antibody
B. Anti-GBM antibody
C. Serum urate
D. HLA-B27
E. Anti-CCP antibody
Answer: B. Anti-GBM disease is an important cause of pulmonary-renal syndrome and commonly requires urgent plasma exchange plus immunosuppression.
SBA 5: Scleroderma renal crisis
A 38-year-old woman with early diffuse systemic sclerosis develops headache, BP 210/120 mmHg, creatinine rise from 0.8 to 2.4 mg/dL, anemia with schistocytes, and thrombocytopenia. What is the most appropriate initial specific treatment?
A. High-dose prednisolone
B. Immediate ACE inhibitor therapy
C. Calcium supplementation
D. Cyclophosphamide alone
E. Stop all antihypertensive medication until renal biopsy
B. Immediate ACE inhibitor therapy
C. Calcium supplementation
D. Cyclophosphamide alone
E. Stop all antihypertensive medication until renal biopsy
Answer: B. This is scleroderma renal crisis. Start an ACE inhibitor urgently.
SBA 6: CAPS
A patient with known APS develops acute kidney injury, livedo reticularis, confusion, thrombocytopenia, and respiratory failure over 4 days following sepsis. Imaging confirms renal and pulmonary thromboses. What is the most likely diagnosis?
A. TTP
B. Disseminated intravascular coagulation only
C. Catastrophic antiphospholipid syndrome
D. Isolated lupus flare
E. Immune thrombocytopenia
B. Disseminated intravascular coagulation only
C. Catastrophic antiphospholipid syndrome
D. Isolated lupus flare
E. Immune thrombocytopenia
Answer: C. Rapid thrombosis involving multiple organs in a patient with APS is CAPS.
SBA 7: CAPS treatment
Which combination best represents first-line treatment for CAPS, in addition to treating the precipitating factor?
A. NSAID + hydroxychloroquine + methotrexate
B. Therapeutic anticoagulation + glucocorticoids + plasma exchange and/or IVIG
C. Aspirin alone
D. Warfarin started without heparin
E. Tocilizumab monotherapy
B. Therapeutic anticoagulation + glucocorticoids + plasma exchange and/or IVIG
C. Aspirin alone
D. Warfarin started without heparin
E. Tocilizumab monotherapy
Answer: B.
SBA 8: MAS
A patient with adult-onset Still disease develops persistent fever, hepatosplenomegaly, falling platelet count, elevated AST, ferritin 38,000 micrograms/L, triglycerides 4.5 mmol/L, and low fibrinogen. What is the diagnosis?
A. Uncomplicated Still disease flare
B. Disseminated gonococcal infection
C. Macrophage activation syndrome
D. Antiphospholipid syndrome
E. Gout
B. Disseminated gonococcal infection
C. Macrophage activation syndrome
D. Antiphospholipid syndrome
E. Gout
Answer: C. The combination of fever, cytopenias, liver dysfunction, hyperferritinemia and hypofibrinogenemia is classic for MAS/secondary HLH.
SBA 9: MAS laboratory clue
Which laboratory trend is particularly concerning for MAS in a patient with systemic JIA or Still disease?
A. Rising fibrinogen
B. Falling ferritin
C. Falling platelet count with rising ferritin and decreasing fibrinogen
D. Isolated positive rheumatoid factor
E. Hypercalcemia
B. Falling ferritin
C. Falling platelet count with rising ferritin and decreasing fibrinogen
D. Isolated positive rheumatoid factor
E. Hypercalcemia
Answer: C.
SBA 10: RA cervical spine
A patient with long-standing rheumatoid arthritis requires emergency intubation after trauma. She has neck pain and hand weakness. Which is the most important precaution?
A. Forceful neck extension for direct laryngoscopy
B. Assume neck pain is due to osteoarthritis
C. Maintain cervical precautions and assess for atlanto-axial instability
D. Perform lumbar puncture before imaging
E. Give intra-articular steroid injection
B. Assume neck pain is due to osteoarthritis
C. Maintain cervical precautions and assess for atlanto-axial instability
D. Perform lumbar puncture before imaging
E. Give intra-articular steroid injection
Answer: C. Atlanto-axial subluxation can result in cord injury during airway manipulation.
SBA 11: SLE and acute dyspnea
A woman with SLE has acute dyspnea, hypoxemia, diffuse bilateral infiltrates, and rapidly falling hemoglobin. There is no overt hemoptysis. What is the most important diagnosis to consider?
A. Pulmonary edema only
B. Diffuse alveolar hemorrhage
C. Panic attack
D. Isolated pleuritis
E. Fibromyalgia
B. Diffuse alveolar hemorrhage
C. Panic attack
D. Isolated pleuritis
E. Fibromyalgia
Answer: B. Hemoptysis can be absent in diffuse alveolar hemorrhage.
SBA 12: General emergency principle
Which statement is most accurate in a patient with established rheumatic disease who develops fever and worsening organ dysfunction?
A. It is usually an autoimmune flare
B. Immunosuppression should always be immediately escalated
C. Infection, thrombosis, drug toxicity, and disease flare must all be assessed urgently
D. ESR alone distinguishes flare from infection
E. Negative blood cultures exclude serious infection
B. Immunosuppression should always be immediately escalated
C. Infection, thrombosis, drug toxicity, and disease flare must all be assessed urgently
D. ESR alone distinguishes flare from infection
E. Negative blood cultures exclude serious infection
Answer: C.
Viva question set with model answers
Viva 1: “List rheumatological emergencies.”
Model answer
I would classify them as:
- Infective: septic arthritis, sepsis or opportunistic infection in an immunosuppressed patient.
- Vascular or thrombotic: giant cell arteritis with visual threat, catastrophic APS, critical digital or mesenteric ischemia.
- Vasculitic organ-threatening disease: pulmonary-renal syndrome, diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis, CNS vasculitis.
- Connective-tissue disease emergencies: scleroderma renal crisis, severe lupus nephritis, neuropsychiatric lupus, alveolar hemorrhage.
- Hyperinflammatory syndromes: MAS/secondary HLH.
- Mechanical or neuromuscular: RA atlanto-axial instability, inflammatory myopathy with bulbar or respiratory weakness.
- Treatment-related: methotrexate toxicity, neutropenic sepsis, serious biologic-associated infection.
Viva 2: “How would you assess a patient with suspected rheumatological emergency?”
Model answer
- Use ABCDE and identify sepsis, shock, hypoxia, encephalopathy, visual loss, AKI, bleeding, and thrombosis.
- Take focused history: rheumatic diagnosis, immunosuppression, recent infection, medication exposure, thrombosis history, pregnancy, and recent procedures.
- Examine for hot joint, rash/purpura, digital ischemia, neurological deficit, lung hemorrhage, hypertension, and ocular signs.
- Request CBC, CRP/ESR, renal/liver profile, urinalysis, coagulation profile, cultures, blood film, ferritin/triglycerides/fibrinogen if MAS is possible, and disease-directed serology.
- Involve ICU and relevant specialists early.
- Do not assume flare until infection and thrombosis are considered.
Viva 3: “How do you manage suspected septic arthritis?”
Model answer
- Regard it as a joint-threatening emergency.
- Assess for sepsis and take blood cultures.
- Aspirate the affected joint urgently for cell count, Gram stain, culture and crystals.
- Begin empiric IV antibiotics after cultures, or immediately if the patient is septic and sampling would delay treatment.
- Obtain urgent orthopedic input for drainage, arthroscopy, arthrotomy, or serial aspiration.
- Tailor antibiotics to microbiological results.
- Remember that crystals and a negative Gram stain do not rule out infection.
Viva 4: “What is your management of suspected GCA with visual symptoms?”
Model answer
- This is a visual emergency.
- Start high-dose glucocorticoids immediately. If there is threatened or established visual loss, use IV pulse glucocorticoids according to local protocol and specialist advice.
- Arrange urgent ophthalmology and rheumatology assessment.
- Send ESR, CRP, CBC and liver tests.
- Arrange temporal artery ultrasound and/or biopsy, but do not delay treatment.
- Assess for large-vessel disease and provide steroid toxicity prevention during ongoing therapy.
Viva 5: “Define pulmonary-renal syndrome and outline management.”
Model answer
Pulmonary-renal syndrome is diffuse alveolar hemorrhage with glomerulonephritis. Key causes are ANCA-associated vasculitis, anti-GBM disease and SLE.
Management includes:
- ICU assessment and respiratory support.
- CBC, renal function, urine microscopy, ANCA, anti-GBM antibodies, complement and lupus serology.
- Chest imaging and consideration of bronchoscopy when appropriate.
- Urgent nephrology and rheumatology input.
- High-dose glucocorticoids and disease-specific induction therapy.
- Anti-GBM disease generally requires plasma exchange in addition to immunosuppression.
- Exclude infection concurrently.
Viva 6: “Describe scleroderma renal crisis.”
Model answer
It is an acute complication of systemic sclerosis characterized by abrupt severe hypertension, AKI, and sometimes microangiopathic hemolytic anemia and thrombocytopenia.
Risk factors include early diffuse cutaneous disease, anti-RNA polymerase III antibodies, rapidly progressive skin disease and glucocorticoid exposure.
The treatment is immediate ACE inhibition, close BP and renal monitoring, ICU/nephrology care where required, and dialysis if needed. An early rise in creatinine is not a reason to stop the ACE inhibitor.
Viva 7: “How would you recognize and treat MAS?”
Model answer
Suspect MAS in a patient with Still disease, systemic JIA, SLE, or another inflammatory disorder who has persistent fever, cytopenias, liver dysfunction, coagulopathy, hypertriglyceridemia, low fibrinogen and markedly elevated ferritin.
I would:
- Stabilize and assess for multiorgan failure.
- Search urgently for infection and malignancy.
- Request serial CBC, LFTs, ferritin, triglycerides, fibrinogen, coagulation profile and hemolysis testing.
- Start high-dose glucocorticoids with urgent rheumatology and hematology input.
- Consider anakinra, cyclosporine, IVIG or other targeted therapy based on cause and severity.
Viva 8: “What is catastrophic APS?”
Model answer
CAPS is a rapidly progressive syndrome of widespread small-vessel thrombosis causing multiorgan failure, usually within a week. It is often triggered by infection, surgery, trauma, pregnancy, anticoagulant withdrawal, malignancy, or active SLE.
Management is ICU-based and includes:
- Therapeutic anticoagulation, usually unfractionated heparin
- High-dose glucocorticoids
- Plasma exchange and/or IVIG
- Identification and treatment of triggers
- Cyclophosphamide if CAPS is associated with active SLE.
Make a one-page emergency revision table
Reading File
Finding Sources
Searching PubMed
rheumatologic emergencies
Searching the Web
rheumatologic emergencies septic arthritis giant cell arteritis pulmonary renal syndrome scleroderma renal crisis emergency guidance
Rheumatological Emergencies: One-Page Revision Table
| Emergency | Recognition clues | Key urgent tests | Immediate action | Exam pearl |
|---|---|---|---|---|
| Septic arthritis | Acute hot, swollen, very painful joint with restricted passive movement. Fever may be absent, especially if immunosuppressed. | Urgent joint aspirate: cell count, Gram stain, culture, crystals. Blood cultures. | Manage as sepsis if unwell. Aspirate promptly, then IV antibiotics and urgent orthopedic drainage/washout as indicated. | Crystals do not rule out infection. Negative Gram stain does not exclude it. |
| Giant cell arteritis | Age >50; new headache, scalp tenderness, jaw claudication, visual disturbance or amaurosis fugax, PMR symptoms. | ESR, CRP, CBC, LFTs. Temporal artery ultrasound and/or biopsy. | Give high-dose glucocorticoids immediately. Urgent ophthalmology and rheumatology if visual symptoms. | Do not wait for biopsy or imaging when vision is threatened. |
| Pulmonary-renal syndrome | Dyspnea, hypoxia, diffuse infiltrates, falling Hb, hemoptysis may be absent, AKI with hematuria/RBC casts. | FBC, renal function, urinalysis, ANCA, anti-GBM, ANA/dsDNA, complement, chest imaging. | ICU/respiratory support if needed; urgent renal-rheumatology review; immunosuppression guided by cause after infection assessment. | Think AAV, anti-GBM disease, or SLE. |
| Diffuse alveolar hemorrhage | Acute breathlessness, hypoxia, falling Hb, bilateral infiltrates, with or without hemoptysis. | Serial Hb, CXR/CT, renal screen, autoimmune serology; bronchoscopy if appropriate. | ICU-level care if severe; treat underlying vasculitis/SLE urgently; exclude infection. | Hemoptysis can be absent. |
| ANCA vasculitis with organ threat | Rapid AKI, pulmonary hemorrhage, purpura, neuropathy, scleritis, ischemic gut or digital ischemia. | Urine microscopy, creatinine, ANCA, anti-GBM, complements; biopsy when safe. | High-dose glucocorticoids plus rituximab or cyclophosphamide under specialist care. | Organ-threatening disease needs urgent induction, not outpatient work-up. |
| Scleroderma renal crisis | Systemic sclerosis with abrupt severe hypertension, AKI, headache, heart failure, MAHA/thrombocytopenia. | Frequent BP, creatinine, urinalysis, FBC, blood film, LDH, haptoglobin. | Start ACE inhibitor immediately, commonly rapidly titratable captopril. Involve nephrology. | Do not stop ACE inhibitor solely because creatinine initially rises. |
| Catastrophic APS | Rapid thromboses in 3 or more organs over days: renal, CNS, pulmonary, skin, cardiac, GI. Often triggered by infection. | FBC, coagulation, D-dimer, renal/liver profile, antiphospholipid antibodies; image thromboses. | ICU care; therapeutic heparin + glucocorticoids + plasma exchange and/or IVIG; treat trigger. | Consider cyclophosphamide if associated with active SLE. |
| Macrophage activation syndrome / secondary HLH | Persistent fever, hepatosplenomegaly, cytopenias, hepatitis, coagulopathy, marked hyperferritinemia. | Ferritin, FBC, LFT, triglycerides, fibrinogen, coagulation, infection and malignancy screen. | Urgent rheumatology, hematology and ICU input; high-dose glucocorticoids and targeted immunomodulation. | High ferritin + falling platelets + low fibrinogen is a key pattern. |
| Severe SLE | Lupus nephritis with AKI, pulmonary hemorrhage, seizures/psychosis, myocarditis, severe cytopenias. | Renal profile, urinalysis, complements, anti-dsDNA, hemolysis screen, APS testing, infection screen. | Stabilize affected organ; distinguish flare from infection, APS and drug toxicity; specialist-directed immunosuppression. | In SLE, not every deterioration is a flare. |
| Inflammatory myopathy crisis | Rapid proximal weakness, dysphagia, aspiration, weak cough, respiratory failure, rhabdomyolysis. | CK, electrolytes, creatinine, ABG, FVC/respiratory measures, ECG/troponin if cardiac concern. | Respiratory and swallow assessment, aspiration precautions, ICU if weakness is severe; treat confirmed inflammatory disease. | Dysphagia may be life-threatening due to aspiration. |
| RA cervical spine instability | Long-standing RA with neck/occipital pain, myelopathy, weakness, gait change or symptoms after minor trauma. | Cervical CT/MRI. | Immobilize, urgent spinal review, alert anesthesia before airway intervention. | Avoid forceful neck extension during intubation. |
| Immunosuppression-related infection/toxicity | Fever, hypotension, neutropenia, mucositis, pancytopenia, atypical/opportunistic infection. | Cultures, FBC, renal/liver profile, imaging directed by symptoms. | Sepsis management, antimicrobials, withhold immunosuppression in serious infection unless specialist-directed. | Biologics and steroids can mask fever and inflammatory response. |
Universal emergency rule
ABCDE first. In any deteriorating rheumatology patient, urgently consider:
Infection
Thrombosis
Flare
Drug toxicity
Thrombosis
Flare
Drug toxicity
Useful first-line tests: FBC, CRP/ESR, renal/liver profile, urinalysis, cultures, coagulation profile, and targeted imaging/serology.
Current guidance supports urgent aspiration and source control in suspected septic arthritis, immediate steroid treatment in suspected GCA with visual risk, and immediate ACE inhibition in scleroderma renal crisis. See the SANJO septic arthritis guideline, ACR GCA guidance, and the 2024 BSR systemic sclerosis guideline.
Turn this table into a printable PDF
Reading File
Reading File
Browsing Directory
~/
Running Command
mkdir -p "/tmp/workspace/rheumatology-emergency-revision/output" [38;5;244m&&[0m python - <<'PY' from reportlab.lib import colors from reportlab.lib.pagesizes import A4, landscape from reportlab.lib.styles import getSampleStyleSheet, ParagraphStyle from reportlab.lib.enums import TA_CENTER from reportlab.lib.units import mm from reportlab.platypus import SimpleDocTemplate, Table, TableStyle, Paragraph, Spacer, KeepTogether from reportlab.pdfbase.pdfmetrics import stringWidth from reportlab.pdfbase.ttfonts import TTFont from reportlab.pdfbase import pdfmetrics from reportlab.lib.colors import HexColor from reportlab.pdfgen import canvas from reportlab.lib import utils from pathlib import Path out = Path('/tmp/workspace/rheumatology-emergency-revision/output/Rheumatological_Emergencies_One_Page_Revision.pdf') PAGE = landscape(A4) W,H = PAGE class NumberedCanvas(canvas.Canvas): def __init__(self,*args,**kwargs): canvas.Canvas.__init__(self,*args,**kwargs) self._saved_page_states=[] def showPage(self): self._saved_page_states.append(dict(self.__dict__)) self._startPage() def save(self): pages=len(self._saved_page_states) for state in self._saved_page_states: self.__dict__.update(state) self.setFont('Helvetica',5.8) self.setFillColor(HexColor('#52616B')) self.drawRightString(W-8*mm,5.5*mm,f'Rheumatological Emergencies | Page {self._pageNumber} of {pages}') self.showPage() canvas.Canvas.save(self) styles=getSampleStyleSheet() base=ParagraphStyle('base', parent=styles['Normal'], fontName='Helvetica', fontSize=5.75, leading=6.55, textColor=HexColor('#111827'), spaceAfter=0) small=ParagraphStyle('small', parent=base, fontSize=5.2, leading=5.9) head=ParagraphStyle('head', parent=base, fontName='Helvetica-Bold', fontSize=6.35, leading=7.2, alignment=TA_CENTER, textColor=colors.white) title=ParagraphStyle('title', parent=styles['Title'], fontName='Helvetica-Bold', fontSize=16, leading=17, alignment=TA_CENTER, textColor=HexColor('#0F3D4C')) sub=ParagraphStyle('sub', parent=base, fontName='Helvetica-Bold', fontSize=7, leading=8, alignment=TA_CENTER, textColor=HexColor('#425466')) call=ParagraphStyle('call', parent=base, fontName='Helvetica-Bold', fontSize=6.35, leading=7.3, textColor=HexColor('#7C2D12')) P=lambda text, style=base: Paragraph(text,style) rows=[ ['<b>Emergency</b>','<b>Recognition clues</b>','<b>Urgent tests</b>','<b>Immediate action</b>','<b>Exam pearl</b>'], ['Septic arthritis','Acute hot, swollen, exquisitely painful joint; passive movement limited. Fever may be absent.','<b>Joint aspirate:</b> cells, Gram stain, culture, crystals. Blood cultures.','Sepsis care if unwell. Aspirate promptly; IV antimicrobials after sampling unless this delays resuscitation. Urgent orthopaedic source control.','Crystals and a negative Gram stain <b>do not</b> exclude infection.'], ['GCA','Age >50, new headache, scalp tenderness, jaw claudication, visual symptoms, PMR.','ESR/CRP, FBC, LFT. Temporal artery ultrasound and/or biopsy.','<b>Immediate high-dose glucocorticoids.</b> Urgent ophthalmology and rheumatology for visual symptoms.','Never wait for biopsy/imaging if vision is threatened.'], ['Pulmonary-renal syndrome / DAH','Dyspnoea, hypoxia, diffuse infiltrates, falling Hb; haemoptysis may be absent. AKI, haematuria/RBC casts.','FBC, renal function, urine microscopy, ANCA, anti-GBM, ANA/dsDNA, complement, CXR/CT.','ICU/respiratory support if severe. Urgent renal-rheumatology input; cause-specific induction after infection assessment.','Think <b>AAV, anti-GBM, SLE.</b>'], ['Organ-threatening vasculitis','Rapid AKI, pulmonary haemorrhage, purpura, neuropathy, scleritis, gut/digital ischaemia.','Urine microscopy, creatinine, ANCA/anti-GBM, complements; biopsy if safe.','High-dose glucocorticoids plus rituximab or cyclophosphamide under specialist care.','Urgent induction, not outpatient work-up.'], ['Scleroderma renal crisis','SSc + abrupt severe HTN, AKI, headache, heart failure, MAHA/thrombocytopenia.','Frequent BP, creatinine, urinalysis, FBC/film, LDH, haptoglobin.','<b>Start ACE inhibitor immediately</b>, often rapidly titratable captopril. Involve nephrology.','Do not stop ACE inhibitor solely for initial creatinine rise.'], ['Catastrophic APS','Multiorgan thromboses over days: renal, CNS, pulmonary, skin, cardiac, GI. Often infection-triggered.','FBC, coagulation, D-dimer, renal/liver profile, aPL antibodies; image thromboses.','ICU; therapeutic heparin + glucocorticoids + plasma exchange and/or IVIG. Treat trigger.','Consider cyclophosphamide with active SLE.'], ['MAS / secondary HLH','Persistent fever, hepatosplenomegaly, cytopenias, hepatitis, coagulopathy, very high ferritin.','Ferritin, FBC, LFT, triglycerides, fibrinogen, coagulation; infection/malignancy screen.','Urgent rheumatology, haematology and ICU; high-dose glucocorticoids plus targeted therapy.','<b>High ferritin + falling platelets + low fibrinogen.</b>'], ['Severe SLE','AKI/nephritis, DAH, seizures/psychosis, myocarditis or severe cytopenias.','Renal profile, urine, complements, dsDNA, haemolysis screen, APS testing, infection screen.','Stabilise organ; distinguish flare from infection, APS and drug toxicity; specialist immunosuppression.','Not every deterioration is a lupus flare.'], ['Inflammatory myopathy crisis','Rapid weakness, dysphagia/aspiration, weak cough, respiratory failure, rhabdomyolysis.','CK, electrolytes/creatinine, ABG, FVC, ECG/troponin if cardiac concern.','Respiratory and swallow assessment; aspiration precautions; ICU for severe weakness.','Bulbar weakness can be fatal.'], ['RA cervical instability','Long-standing RA + neck/occipital pain, myelopathy, weakness, gait change.','Cervical CT/MRI.','Immobilise, urgent spinal review; tell anaesthesia before airway intervention.','Avoid forceful neck extension at intubation.'], ['Immunosuppression infection/toxicity','Fever/shock, neutropenia, mucositis, pancytopenia, atypical opportunistic infection.','Cultures, FBC, renal/liver profile, focused imaging.','Sepsis care and antimicrobials; withhold immunosuppression in serious infection unless advised.','Steroids/biologics can mask fever and CRP response.'], ] # Convert rows into compact paragraphs pdata=[] for ri,row in enumerate(rows): st=head if ri==0 else base pdata.append([P(c, st if ri==0 else base) for c in row]) colwidths=[29*mm,55*mm,46*mm,72*mm,47*mm] t=Table(pdata, colWidths=colwidths, repeatRows=1, hAlign='CENTER') t.setStyle(TableStyle([ ('BACKGROUND',(0,0),(-1,0),HexColor('#0F5B78')),('TEXTCOLOR',(0,0),(-1,0),colors.white), ('VALIGN',(0,0),(-1,-1),'TOP'),('GRID',(0,0),(-1,-1),0.25,HexColor('#B8C5CC')), ('LEFTPADDING',(0,0),(-1,-1),2.0),('RIGHTPADDING',(0,0),(-1,-1),2.0),('TOPPADDING',(0,0),(-1,-1),1.6),('BOTTOMPADDING',(0,0),(-1,-1),1.6), ('BACKGROUND',(0,1),(-1,-1),HexColor('#F8FBFC')), ('BACKGROUND',(0,2),(-1,2),HexColor('#EEF6F8')), ('BACKGROUND',(0,4),(-1,4),HexColor('#EEF6F8')), ('BACKGROUND',(0,6),(-1,6),HexColor('#EEF6F8')), ('BACKGROUND',(0,8),(-1,8),HexColor('#EEF6F8')), ('BACKGROUND',(0,10),(-1,10),HexColor('#EEF6F8')), ('FONTNAME',(0,1),(0,-1),'Helvetica-Bold'), ])) story=[Spacer(1,4*mm), P('RHEUMATOLOGICAL EMERGENCIES',title), Spacer(1,1.0*mm), P('ONE-PAGE EXAM REVISION TABLE',sub), Spacer(1,3*mm), t, Spacer(1,2.2*mm)] alert_data=[[P('<b>UNIVERSAL RULE: ABCDE first.</b> In any deteriorating rheumatology patient, urgently assess for <b>I</b>nfection, <b>T</b>hrombosis, <b>F</b>lare and <b>D</b>rug toxicity. First-line tests: FBC, CRP/ESR, renal/liver profile, urinalysis, cultures, coagulation profile and targeted imaging/serology.', call)]] a=Table(alert_data,colWidths=[249*mm]) a.setStyle(TableStyle([('BACKGROUND',(0,0),(-1,-1),HexColor('#FFF7ED')),('BOX',(0,0),(-1,-1),0.5,HexColor('#FB923C')),('LEFTPADDING',(0,0),(-1,-1),4),('RIGHTPADDING',(0,0),(-1,-1),4),('TOPPADDING',(0,0),(-1,-1),3),('BOTTOMPADDING',(0,0),(-1,-1),3)])) story.append(a) story.append(Spacer(1,1.2*mm)) story.append(P('For examination revision only. Drug selection, dose and escalation must follow local protocol and specialist advice. Content reflects guideline-oriented summary discussed in this conversation.', small)) doc=SimpleDocTemplate(str(out),pagesize=PAGE,leftMargin=8*mm,rightMargin=8*mm,topMargin=5*mm,bottomMargin=9*mm,title='Rheumatological Emergencies - One-Page Revision',author='Orris') doc.build(story, canvasmaker=NumberedCanvas) print(out) PY python - <<'PY' from pypdf import PdfReader p='/tmp/workspace/rheumatology-emergency-revision/output/Rheumatological_Emergencies_One_Page_Revision.pdf' r=PdfReader(p) print('pages=',len(r.pages),'size=',__import__('os').path.getsize(p)) print('text_sample=',r.pages[0].extract_text()[:180].replace('\n',' | ')) PY
Browsing Directory
~/
Writing File
~/rheumatology-emergency-revision/create_pdf.py
from pathlib import Path
from reportlab.lib import colors
from reportlab.lib.pagesizes import A4, landscape
from reportlab.lib.styles import getSampleStyleSheet, ParagraphStyle
from reportlab.lib.enums import TA_CENTER
from reportlab.lib.units import mm
from reportlab.platypus import SimpleDocTemplate, Table, TableStyle, Paragraph, Spacer
from reportlab.pdfgen import canvas
from reportlab.lib.colors import HexColor
OUT_DIR = Path('/tmp/workspace/rheumatology-emergency-revision/output')
OUT_DIR.mkdir(parents=True, exist_ok=True)
OUT = OUT_DIR / 'Rheumatological_Emergencies_One_Page_Revision.pdf'
PAGE = landscape(A4)
W, H = PAGE
class NumberedCanvas(canvas.Canvas):
def __init__(self, *args, **kwargs):
super().__init__(*args, **kwargs)
self.saved_states = []
def showPage(self):
self.saved_states.append(dict(self.__dict__))
self._startPage()
def save(self):
total = len(self.saved_states)
for state in self.saved_states:
self.__dict__.update(state)
self.setFont('Helvetica', 5.8)
self.setFillColor(HexColor('#52616B'))
self.drawRightString(W - 8*mm, 5.2*mm, f'Rheumatological Emergencies | Page {self._pageNumber} of {total}')
super().showPage()
super().save()
styles = getSampleStyleSheet()
base = ParagraphStyle('base', parent=styles['Normal'], fontName='Helvetica', fontSize=5.65, leading=6.35, textColor=HexColor('#111827'))
head = ParagraphStyle('head', parent=base, fontName='Helvetica-Bold', fontSize=6.25, leading=7.0, alignment=TA_CENTER, textColor=colors.white)
title = ParagraphStyle('title', parent=styles['Title'], fontName='Helvetica-Bold', fontSize=16, leading=17, alignment=TA_CENTER, textColor=HexColor('#0F3D4C'))
sub = ParagraphStyle('sub', parent=base, fontName='Helvetica-Bold', fontSize=7, leading=8, alignment=TA_CENTER, textColor=HexColor('#425466'))
call = ParagraphStyle('call', parent=base, fontName='Helvetica-Bold', fontSize=6.25, leading=7.1, textColor=HexColor('#7C2D12'))
foot = ParagraphStyle('foot', parent=base, fontSize=5.1, leading=5.7, textColor=HexColor('#52616B'))
P = lambda text, style=base: Paragraph(text, style)
rows = [
['Emergency', 'Recognition clues', 'Urgent tests', 'Immediate action', 'Exam pearl'],
['Septic arthritis', 'Acute hot, swollen, exquisitely painful joint; passive movement limited. Fever may be absent.', '<b>Joint aspirate:</b> cells, Gram stain, culture, crystals. Blood cultures.', 'Sepsis care if unwell. Aspirate promptly; IV antimicrobials after sampling unless this delays resuscitation. Urgent orthopaedic source control.', 'Crystals and a negative Gram stain <b>do not</b> exclude infection.'],
['GCA', 'Age >50, new headache, scalp tenderness, jaw claudication, visual symptoms, PMR.', 'ESR/CRP, FBC, LFT. Temporal artery ultrasound and/or biopsy.', '<b>Immediate high-dose glucocorticoids.</b> Urgent ophthalmology and rheumatology for visual symptoms.', 'Never wait for biopsy/imaging if vision is threatened.'],
['Pulmonary-renal syndrome / DAH', 'Dyspnoea, hypoxia, diffuse infiltrates, falling Hb; haemoptysis may be absent. AKI, haematuria/RBC casts.', 'FBC, renal function, urine microscopy, ANCA, anti-GBM, ANA/dsDNA, complement, CXR/CT.', 'ICU/respiratory support if severe. Urgent renal-rheumatology input; cause-specific induction after infection assessment.', 'Think <b>AAV, anti-GBM, SLE.</b>'],
['Organ-threatening vasculitis', 'Rapid AKI, pulmonary haemorrhage, purpura, neuropathy, scleritis, gut/digital ischaemia.', 'Urine microscopy, creatinine, ANCA/anti-GBM, complements; biopsy if safe.', 'High-dose glucocorticoids plus rituximab or cyclophosphamide under specialist care.', 'Urgent induction, not outpatient work-up.'],
['Scleroderma renal crisis', 'SSc + abrupt severe HTN, AKI, headache, heart failure, MAHA/thrombocytopenia.', 'Frequent BP, creatinine, urinalysis, FBC/film, LDH, haptoglobin.', '<b>Start ACE inhibitor immediately</b>, often rapidly titratable captopril. Involve nephrology.', 'Do not stop ACE inhibitor solely for initial creatinine rise.'],
['Catastrophic APS', 'Multiorgan thromboses over days: renal, CNS, pulmonary, skin, cardiac, GI. Often infection-triggered.', 'FBC, coagulation, D-dimer, renal/liver profile, aPL antibodies; image thromboses.', 'ICU; therapeutic heparin + glucocorticoids + plasma exchange and/or IVIG. Treat trigger.', 'Consider cyclophosphamide with active SLE.'],
['MAS / secondary HLH', 'Persistent fever, hepatosplenomegaly, cytopenias, hepatitis, coagulopathy, very high ferritin.', 'Ferritin, FBC, LFT, triglycerides, fibrinogen, coagulation; infection/malignancy screen.', 'Urgent rheumatology, haematology and ICU; high-dose glucocorticoids plus targeted therapy.', '<b>High ferritin + falling platelets + low fibrinogen.</b>'],
['Severe SLE', 'AKI/nephritis, DAH, seizures/psychosis, myocarditis or severe cytopenias.', 'Renal profile, urine, complements, dsDNA, haemolysis screen, APS testing, infection screen.', 'Stabilise organ; distinguish flare from infection, APS and drug toxicity; specialist immunosuppression.', 'Not every deterioration is a lupus flare.'],
['Inflammatory myopathy crisis', 'Rapid weakness, dysphagia/aspiration, weak cough, respiratory failure, rhabdomyolysis.', 'CK, electrolytes/creatinine, ABG, FVC, ECG/troponin if cardiac concern.', 'Respiratory and swallow assessment; aspiration precautions; ICU for severe weakness.', 'Bulbar weakness can be fatal.'],
['RA cervical instability', 'Long-standing RA + neck/occipital pain, myelopathy, weakness, gait change.', 'Cervical CT/MRI.', 'Immobilise, urgent spinal review; tell anaesthesia before airway intervention.', 'Avoid forceful neck extension at intubation.'],
['Immunosuppression infection/toxicity', 'Fever/shock, neutropenia, mucositis, pancytopenia, atypical opportunistic infection.', 'Cultures, FBC, renal/liver profile, focused imaging.', 'Sepsis care and antimicrobials; withhold immunosuppression in serious infection unless advised.', 'Steroids/biologics can mask fever and CRP response.'],
]
data = []
for r, row in enumerate(rows):
data.append([P(x, head if r == 0 else base) for x in row])
col_widths = [29*mm, 55*mm, 46*mm, 72*mm, 47*mm]
table = Table(data, colWidths=col_widths, repeatRows=1)
table.setStyle(TableStyle([
('BACKGROUND', (0,0), (-1,0), HexColor('#0F5B78')),
('TEXTCOLOR', (0,0), (-1,0), colors.white),
('VALIGN', (0,0), (-1,-1), 'TOP'),
('GRID', (0,0), (-1,-1), 0.25, HexColor('#B8C5CC')),
('LEFTPADDING', (0,0), (-1,-1), 2.0), ('RIGHTPADDING', (0,0), (-1,-1), 2.0),
('TOPPADDING', (0,0), (-1,-1), 1.55), ('BOTTOMPADDING', (0,0), (-1,-1), 1.55),
('BACKGROUND', (0,1), (-1,-1), HexColor('#F8FBFC')),
('BACKGROUND', (0,2), (-1,2), HexColor('#EEF6F8')),
('BACKGROUND', (0,4), (-1,4), HexColor('#EEF6F8')),
('BACKGROUND', (0,6), (-1,6), HexColor('#EEF6F8')),
('BACKGROUND', (0,8), (-1,8), HexColor('#EEF6F8')),
('BACKGROUND', (0,10), (-1,10), HexColor('#EEF6F8')),
('FONTNAME', (0,1), (0,-1), 'Helvetica-Bold'),
]))
alert = Table([[P('<b>UNIVERSAL RULE: ABCDE first.</b> In any deteriorating rheumatology patient, urgently assess for <b>I</b>nfection, <b>T</b>hrombosis, <b>F</b>lare and <b>D</b>rug toxicity. First-line tests: FBC, CRP/ESR, renal/liver profile, urinalysis, cultures, coagulation profile and targeted imaging/serology.', call)]], colWidths=[249*mm])
alert.setStyle(TableStyle([
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Your credits are exhausted. You can purchase a top-up or subscribe to a plan to continue using Orris. If you're already subscribed, your daily credits will reset tomorrow.## Rheumatological emergencies: topic map
-
Acute crystal arthritis
- Septic arthritis must be excluded before assuming gout or CPPD.
- Arthrocentesis, microscopy, Gram stain and culture are central.
-
Septic arthritis
- A rapidly destructive joint infection requiring urgent aspiration, antibiotics and often surgical drainage.
-
Acute spinal emergencies
- Atlanto-axial subluxation and cervical myelopathy in rheumatoid arthritis.
- Spinal epidural abscess can mimic an inflammatory flare.
-
Systemic vasculitis emergencies
- Pulmonary-renal syndrome, diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis.
- Mesenteric ischemia, mononeuritis multiplex, and critical limb ischemia.
-
Giant cell arteritis
- Threatened or established visual loss requires immediate glucocorticoids, without awaiting biopsy or imaging.
-
Catastrophic antiphospholipid syndrome
- Acute thromboses in multiple organs, often with thrombocytopenia and microangiopathy.
-
Systemic lupus erythematosus emergencies
- Lupus nephritis with rapidly deteriorating renal function, neuropsychiatric lupus, severe cytopenias, myocarditis, pulmonary hemorrhage.
-
Scleroderma renal crisis
- Abrupt hypertension and acute kidney injury, managed urgently with an ACE inhibitor.
-
Inflammatory myopathy emergencies
- Respiratory-muscle weakness, severe dysphagia with aspiration, and rhabdomyolysis.
-
Macrophage activation syndrome / secondary HLH
- Persistent high fever, cytopenias, liver dysfunction, coagulopathy and markedly high ferritin, often complicating systemic JIA or adult-onset Still disease.
-
Drug and treatment-related emergencies
- Serious infection in immunosuppressed patients, neutropenic sepsis, infusion reactions, and medication toxicities.
High-level approach
A rheumatological emergency is usually one of three patterns: infection mistaken for inflammation, organ-threatening immune disease, or thrombotic/vascular occlusion. Initial management prioritizes ABCDE assessment, sepsis evaluation, urgent specialist involvement, and targeted tests such as joint aspiration, urinalysis with renal function, full blood count, coagulation tests, inflammatory markers, cultures, chest imaging, and autoimmune studies where appropriate.
Do not delay time-critical treatment when there is suspected bacterial sepsis, threatened vision in giant cell arteritis, pulmonary hemorrhage, rapidly progressive renal failure, hypertensive renal crisis, or multi-organ thrombosis.
Which topic should I cover in detail?
- Exam-oriented complete outline
- Emergency recognition and initial management
- Vasculitis and pulmonary-renal syndromes
- SLE, APS, scleroderma and MAS# Rheumatological Emergencies: Exam-Oriented Complete Outline
Definition
Rheumatological emergencies are conditions related to inflammatory, autoimmune, crystal, thrombotic, infective, or treatment-related rheumatic disease that can cause rapid organ loss, irreversible disability, or death without urgent recognition and treatment.
Universal approach in the emergency setting
1. Recognize red flags
- Fever, hypotension, tachycardia, altered sensorium
- Acutely red, hot, swollen joint
- Visual symptoms or new temporal headache
- Hemoptysis, hypoxia, falling hemoglobin
- Acute kidney injury, hematuria, proteinuria, hypertension
- Purpura, digital ischemia, mononeuritis multiplex
- Cytopenias, hepatitis, coagulopathy, hyperferritinemia
- Severe muscle weakness, dysphagia, respiratory weakness
- Multi-organ thrombosis
2. Initial investigations
- CBC with differential and platelet count
- ESR, CRP, renal and liver function tests
- Urinalysis and urine microscopy
- Blood cultures before antibiotics if sepsis is possible
- Coagulation profile, LDH, haptoglobin, blood film where thrombotic microangiopathy is possible
- Creatine kinase in suspected myositis/rhabdomyolysis
- Ferritin, triglycerides, fibrinogen in suspected MAS/HLH
- Chest radiograph or CT chest for pulmonary involvement
- Autoimmune tests guided by syndrome: ANA, dsDNA, complement, ANCA, anti-GBM, antiphospholipid antibodies
- Urgent specialist input: rheumatology plus the affected organ specialty, ICU, orthopedics, ophthalmology, nephrology, hematology, or respiratory medicine.
3. Core rule
Do not attribute acute deterioration to a rheumatologic flare until infection, thrombosis, drug toxicity, and malignancy have been considered.
1. Septic arthritis
Why it is an emergency
Rapid cartilage destruction, permanent loss of joint function, bacteremia, and death can occur. Fever may be absent, especially in older adults and immunosuppressed patients.
The SANJO guideline states that no clinical parameter alone can confidently rule septic arthritis in or out, and a painful, inflamed joint warrants high suspicion. SANJO guideline
Risk factors
- Rheumatoid arthritis or other chronic arthropathy
- Prosthetic joint
- Immunosuppression, including glucocorticoids and biologics
- Diabetes, chronic kidney disease, cirrhosis
- IV drug use
- Recent joint injection, surgery, skin infection, or bacteremia
Clinical features
- Acute monoarthritis: severe pain, swelling, warmth, restricted active and passive movement
- Knee most common, then hip, shoulder, wrist, ankle
- Fever or systemic toxicity may occur
- Polyarticular disease can occur in rheumatoid arthritis and disseminated gonococcal infection.
Diagnosis
- Urgent arthrocentesis before antibiotics, if this does not delay resuscitation in septic shock.
- Send synovial fluid for:
- Cell count and differential
- Gram stain
- Culture and sensitivity
- Crystal analysis
- Obtain blood cultures.
- A negative Gram stain or crystals in aspirate do not exclude concurrent infection.
Management
- ABCDE and sepsis management.
- Prompt IV empirical antibiotics after cultures.
- Urgent orthopedic consultation for repeated aspiration, arthroscopic washout, or open drainage.
- Treat as septic arthritis until excluded.
Exam pearl: A hot swollen joint in an immunosuppressed patient is septic arthritis until proven otherwise.
2. Giant cell arteritis: visual emergency
Why it is an emergency
GCA can cause sudden irreversible visual loss due to ischemic optic neuropathy. It needs treatment immediately if strongly suspected, without waiting for temporal artery biopsy or imaging.
The American College of Rheumatology notes that treatment should begin immediately to prevent visual loss. ACR GCA guidance
Suspect GCA in a patient aged over 50 with
- New-onset temporal or generalized headache
- Scalp tenderness
- Jaw or tongue claudication
- Visual blurring, diplopia, amaurosis fugax, or visual loss
- Polymyalgia rheumatica symptoms
- Constitutional symptoms
- Raised ESR and CRP, though normal markers do not completely exclude it.
Investigations
- ESR, CRP, CBC, liver function tests
- Temporal artery ultrasound where expertise is available
- Temporal artery biopsy, ideally soon after treatment begins
- Urgent ophthalmology review for visual symptoms.
Management
- No visual ischemia: high-dose oral glucocorticoid therapy, commonly prednisolone 40-60 mg/day.
- Threatened or established visual loss: urgent IV methylprednisolone is generally preferred, followed by high-dose oral glucocorticoids.
- Do not delay steroids for biopsy.
- Long-term care includes gradual tapering, relapse surveillance, steroid-toxicity prevention, and consideration of steroid-sparing treatment such as tocilizumab under specialist care.
Exam pearl: Jaw claudication is highly suggestive; visual symptoms require immediate glucocorticoids.
3. Pulmonary-renal syndrome and diffuse alveolar hemorrhage
Definition
Pulmonary-renal syndrome is diffuse alveolar hemorrhage plus glomerulonephritis. It is most often caused by:
- ANCA-associated vasculitis: GPA or MPA
- Anti-GBM disease
- SLE
- Less often, IgA vasculitis or cryoglobulinemic vasculitis
Presentation
- Dyspnea, cough, hypoxia
- Hemoptysis may be absent
- Falling hemoglobin and diffuse pulmonary infiltrates
- Acute kidney injury
- Hematuria, proteinuria, dysmorphic red cells, red-cell casts
- Hypertension and systemic vasculitic features.
Diffuse alveolar hemorrhage often presents with dyspnea, hemoptysis, alveolar opacity, and anemia, but hemoptysis may be absent. Pulmonary-renal syndrome requires urgent evaluation. Comprehensive Clinical Nephrology, 7th Edition, p. 1381.
Immediate management
- Admit to HDU/ICU if hypoxic or rapidly deteriorating.
- Oxygen and ventilatory support where needed.
- Urgent nephrology and rheumatology input.
- Urinalysis, renal function, ANCA, anti-GBM, complements, ANA/dsDNA.
- Consider bronchoscopy with bronchoalveolar lavage if diagnosis is uncertain and patient is stable enough.
- Renal biopsy is often needed, but do not delay treatment in life-threatening disease.
- High-dose glucocorticoids plus disease-specific immunosuppression, commonly rituximab or cyclophosphamide for severe ANCA vasculitis.
- Anti-GBM disease usually requires plasma exchange plus immunosuppression.
- Exclude infection before escalating immunosuppression.
Exam pearl: Hemoptysis + acute kidney injury + active urinary sediment = pulmonary-renal syndrome until proved otherwise.
4. Systemic vasculitis with organ-threatening disease
Red-flag manifestations
- Rapidly progressive glomerulonephritis
- Alveolar hemorrhage
- Mononeuritis multiplex or acute foot/wrist drop
- Mesenteric ischemia or gastrointestinal perforation
- Digital ischemia or gangrene
- CNS involvement: seizures, stroke, encephalopathy
- Scleritis or retinal vasculitis
- Myocarditis or coronary vasculitis
Key clinical patterns
| Condition | High-yield clues | Emergency risk |
|---|---|---|
| GPA | ENT disease, pulmonary nodules/hemorrhage, glomerulonephritis, PR3-ANCA | Pulmonary-renal syndrome |
| MPA | Pulmonary capillaritis, glomerulonephritis, MPO-ANCA | DAH, rapidly progressive GN |
| PAN | Hypertension, abdominal pain, neuropathy, livedo | Mesenteric or renal ischemia |
| Cryoglobulinemic vasculitis | Purpura, neuropathy, low C4, hepatitis C association | Renal and neurologic involvement |
| Behçet disease | Oral/genital ulcers, uveitis, thrombosis | Cerebral venous thrombosis, arterial aneurysm |
| Takayasu arteritis | Pulse asymmetry, bruits, hypertension | Cerebrovascular or cardiac ischemia |
Principles of management
- Stabilize affected organ system.
- Obtain cultures and infection screen.
- Give high-dose glucocorticoids when life or organ is threatened.
- Add induction immunosuppression under specialist supervision.
- Tissue diagnosis, often kidney, skin, nerve, lung, or temporal artery biopsy, should be pursued when safe but must not delay lifesaving therapy.
5. Scleroderma renal crisis
Definition
An acute, life-threatening complication of systemic sclerosis characterized by:
- Abrupt severe hypertension, sometimes normotensive
- Acute kidney injury
- Thrombotic microangiopathy and microangiopathic hemolytic anemia
- Hypertensive encephalopathy, heart failure, or retinopathy in severe cases
Scleroderma renal crisis presents with accelerated hypertension, acute kidney injury, and progressive renal failure. Harrison's Principles of Internal Medicine, 22nd Edition, p. 2651.
Risk factors
- Early diffuse cutaneous systemic sclerosis
- Rapid skin thickening
- Anti-RNA polymerase III antibodies
- Recent high-dose glucocorticoid exposure
- Tendon friction rubs
Investigations
- BP monitoring, renal function, urinalysis
- CBC, blood film, LDH, bilirubin, haptoglobin
- ECG, echocardiography if heart failure suspected
- Fundoscopy where available.
Management
- Start ACE inhibitor immediately, classically captopril because rapid titration is possible.
- Aggressively control BP with close monitoring.
- Do not stop ACE inhibitor solely because creatinine rises initially.
- Dialysis may be temporarily required.
- Avoid high-dose glucocorticoids unless another compelling indication exists.
Exam pearl: In systemic sclerosis with sudden hypertension and AKI, start an ACE inhibitor urgently.
6. Catastrophic antiphospholipid syndrome
Definition
Catastrophic APS is an accelerated thrombotic storm causing small-vessel thrombosis and rapidly progressive failure of three or more organ systems, typically developing within a week.
Triggers
- Infection
- Surgery or trauma
- Anticoagulation withdrawal
- Pregnancy/postpartum state
- SLE flare
- Malignancy
Clinical features
- Renal failure
- Encephalopathy, stroke, seizures
- Respiratory failure/ARDS
- Myocardial ischemia
- Abdominal pain from mesenteric thrombosis
- Skin necrosis, livedo
- Thrombocytopenia and hemolytic anemia
Investigations
- CBC and blood film
- PT, aPTT, fibrinogen, D-dimer
- Renal and liver function
- Lupus anticoagulant, anticardiolipin, anti-beta-2 glycoprotein I antibodies
- Imaging to identify thrombosis
- Evaluate mimics: DIC, TTP, HUS, HIT, sepsis.
Management
- ICU-level multidisciplinary care.
- Therapeutic anticoagulation, usually IV unfractionated heparin.
- High-dose glucocorticoids.
- Plasma exchange and/or IV immunoglobulin for severe disease.
- Treat trigger, particularly infection.
- Consider cyclophosphamide if CAPS occurs with active SLE.
- Refractory cases require specialist-directed therapies.
Exam pearl: Multiorgan thrombosis over days in a patient with APS is CAPS, not simply recurrent venous thromboembolism.
7. SLE emergencies
A. Lupus nephritis with rapidly progressive renal failure
Features
- Rising creatinine
- Proteinuria, hematuria, red-cell casts
- Hypertension, edema
- Low complement, rising anti-dsDNA may support active lupus.
Management
- Urgent nephrology-rheumatology referral.
- Renal biopsy where possible.
- High-dose glucocorticoids plus induction immunosuppression according to biopsy class and severity.
- Exclude infection, drug toxicity, thrombotic microangiopathy, and APS nephropathy.
B. Diffuse alveolar hemorrhage
- Dyspnea, falling hemoglobin, hypoxia, pulmonary infiltrates.
- Manage in ICU with high-dose glucocorticoids and specialist immunosuppression after infection evaluation.
C. Neuropsychiatric lupus
- Seizures, psychosis, acute confusional state, stroke, myelitis.
- Exclude infection, metabolic disease, drug effects, thrombosis, and posterior reversible encephalopathy syndrome.
- MRI brain/spine, CSF when appropriate, and APS testing.
D. Severe autoimmune cytopenias
- Immune thrombocytopenia with bleeding
- Autoimmune hemolytic anemia
- Neutropenia with sepsis risk
- Treat with glucocorticoids, IVIG in selected urgent cases, transfusion support as clinically indicated.
8. Macrophage activation syndrome and secondary HLH
Definition
MAS is a form of secondary hemophagocytic lymphohistiocytosis caused by uncontrolled immune activation. It is classically associated with systemic JIA, adult-onset Still disease, and SLE.
Clinical features
- Persistent high fever
- Hepatosplenomegaly
- Cytopenias
- Hepatitis, rising transaminases
- Coagulopathy, low fibrinogen
- Hypertriglyceridemia
- Very high ferritin
- Neurologic involvement
- Multiorgan failure
In systemic JIA, ferritin greater than 684 micrograms/L plus any two of thrombocytopenia, raised AST, hypertriglyceridemia, or low fibrinogen supports classification of MAS. Rheumatology, 2-Volume Set, p. 6089.
Important distinction
An apparent fall in ESR in a highly inflamed patient can be ominous because fibrinogen is consumed.
Management
- ICU assessment if unstable.
- Screen aggressively for infection and malignancy.
- High-dose glucocorticoids.
- Specialist-directed anakinra, cyclosporine, IVIG, or other immunomodulation depending on cause and severity.
- Support organ failure and treat triggers.
Exam pearl: Fever + falling cell counts + high ferritin + low fibrinogen in Still disease/systemic JIA strongly suggests MAS.
9. Inflammatory myopathy emergencies
Emergency manifestations
- Respiratory muscle weakness causing type 2 respiratory failure
- Severe bulbar weakness and dysphagia causing aspiration
- Rapidly progressive weakness
- Rhabdomyolysis with acute kidney injury
- Myocarditis or arrhythmia in selected inflammatory myopathies
Assessment
- Forced vital capacity and negative inspiratory force
- Arterial blood gas if respiratory compromise
- Swallow assessment
- CK, electrolytes, renal function, ECG, troponin when cardiac involvement is possible
- Assess for infection and drug-induced myopathy.
Management
- ICU or respiratory support when required.
- Aspiration precautions and enteral feeding plan.
- High-dose glucocorticoids plus early steroid-sparing immunosuppression for severe confirmed inflammatory disease.
- IVIG is particularly useful in severe dysphagia or selected refractory cases.
- Manage rhabdomyolysis with fluids and electrolyte correction.
10. Cervical spine instability in rheumatoid arthritis
Mechanism
Chronic RA may cause atlanto-axial subluxation, basilar invagination, or subaxial cervical instability.
Red flags
- Neck pain or occipital headache
- Myelopathy: gait disturbance, hyperreflexia, weakness
- Paresthesia, sphincter symptoms
- Symptoms after minor trauma
- Difficulty positioning for intubation.
Management
- Immobilize the neck if instability is suspected.
- Urgent CT/MRI and spinal surgical review.
- Inform anesthesiology before airway manipulation.
Exam pearl: Never force neck extension for intubation in RA with suspected cervical spine involvement.
11. Acute crystal arthritis: the septic arthritis trap
Gout and CPPD may cause severe inflammatory monoarthritis but are usually not life-threatening. The emergency is failure to recognize coexistent septic arthritis.
Acute gout
- Abrupt, severe pain, classically first MTP but can involve any joint.
- Needle-shaped, negatively birefringent monosodium urate crystals.
Acute CPPD
- Commonly knee or wrist.
- Rhomboid, weakly positively birefringent crystals.
- Consider metabolic associations in younger patients.
Principle
Perform joint aspiration when infection is plausible. Crystals do not exclude infection.
12. Drug-related emergencies in rheumatology
Serious infection
Patients receiving glucocorticoids, conventional DMARDs, biologics, JAK inhibitors, or cyclophosphamide may have muted fever and inflammatory-marker responses.
- Consider bacterial sepsis, opportunistic infection, tuberculosis, Pneumocystis pneumonia, herpes zoster, and invasive fungal infection according to therapy and risk factors.
- Withhold immunosuppressive therapy in serious infection unless a specialist directs otherwise.
Methotrexate toxicity
- Mucositis, pancytopenia, renal failure, transaminitis.
- Risk increased by renal impairment and interacting medications.
- Urgent blood counts, renal/liver tests, infection assessment, and specialist toxicology/hematology input.
Cyclophosphamide toxicity
- Myelosuppression, hemorrhagic cystitis, infection.
- Neutropenic fever is a medical emergency.
Short-answer exam framework
When asked to list rheumatological emergencies, write:
- Septic arthritis
- Giant cell arteritis with threatened visual loss
- Pulmonary-renal syndrome due to vasculitis, anti-GBM disease, or SLE
- Rapidly progressive glomerulonephritis
- Diffuse alveolar hemorrhage
- Scleroderma renal crisis
- Catastrophic antiphospholipid syndrome
- Macrophage activation syndrome/secondary HLH
- Severe SLE: nephritis, CNS lupus, cytopenias, myocarditis, pulmonary hemorrhage
- Severe systemic vasculitis with organ ischemia
- Inflammatory myopathy with respiratory or bulbar weakness
- RA-related atlanto-axial subluxation
- Serious infection and drug toxicity in immunosuppressed patients
One-line revision points
- Hot swollen joint: aspirate urgently and treat as septic until excluded.
- GCA plus visual symptom: give glucocorticoid immediately.
- Hemoptysis plus AKI: suspect pulmonary-renal syndrome.
- Scleroderma plus hypertension and AKI: start ACE inhibitor urgently.
- APS plus rapid multiorgan thrombosis: treat as CAPS with anticoagulation and immunomodulatory therapy.
- Still disease/systemic JIA plus cytopenias and high ferritin: suspect MAS.
- RA plus neurological signs or neck pain: consider cervical instability.# Latest guideline map for rheumatological emergencies
There is no single guideline covering every rheumatological emergency. In exams and clinical practice, use the most recent disease-specific guideline alongside local sepsis, ICU, renal, and thrombosis protocols.
| Emergency | Most current high-value guideline | Exam-relevant message |
|---|---|---|
| Septic arthritis | GEIO/SEIMC/SEIP/SECOT adult and paediatric guideline, 2024 [PMID: 37919201]; also SANJO guideline, 2023 | Treat a painful, hot, swollen joint as septic until excluded. Aspirate promptly for microscopy, culture and crystals. Obtain blood cultures. Start IV antimicrobials after sampling, unless sepsis mandates immediate treatment. Arrange source control, commonly surgical washout or serial aspiration. |
| Giant cell arteritis with visual symptoms | ACR/Vasculitis Foundation guideline, 2021 [PMID: 34235884]; BSR guideline, 2020 [PMID: 31970405] | GCA is a vision-threatening emergency. Start glucocorticoids immediately when strongly suspected. Do not wait for temporal artery biopsy or imaging. Threatened visual loss generally warrants IV pulse glucocorticoids under urgent specialist care. |
| ANCA-associated vasculitis with pulmonary-renal syndrome or rapidly progressive GN | BSR recommendations, 2025 [PMID: 40499922]; KDIGO guideline, 2024 [PMID: 38388102] | Life- or organ-threatening AAV requires urgent glucocorticoid-based induction plus rituximab or cyclophosphamide, chosen according to clinical setting. Investigate urgently for ANCA, anti-GBM antibody, renal involvement, and infection. |
| Scleroderma renal crisis | BSR systemic sclerosis guideline, 2024 [PMID: 39255973] | Recognize abrupt hypertension with AKI and possible thrombotic microangiopathy. Start an ACE inhibitor immediately and titrate rapidly. Do not withhold ACE inhibition solely because creatinine initially rises. Monitor BP closely in patients exposed to glucocorticoids. |
| Severe SLE including nephritis, alveolar hemorrhage, neuropsychiatric SLE and cytopenias | ACR SLE guideline, 2025 [PMID: 41187097], published 2026; BSR SLE guideline, 2026 [PMID: 42336388] | First distinguish active lupus from infection, thrombotic APS, drug toxicity, and hypertensive emergency. Severe organ involvement requires multidisciplinary care and prompt immunosuppression tailored to organ disease. |
| Macrophage activation syndrome/secondary HLH | HiHASC adult HLH consensus guideline, 2024 [PMID: 38258680] | Think of MAS/HLH in persistent fever with cytopenias, hepatitis, coagulopathy, high ferritin and falling fibrinogen. Simultaneously seek infection, malignancy, and autoimmune triggers. Treat in collaboration with rheumatology, hematology, and ICU. |
| Catastrophic antiphospholipid syndrome | No newer universally adopted CAPS-specific guideline was identified. The commonly cited multidisciplinary framework remains the 2018 McMaster RARE-Best Practices guideline. | Suspect in rapidly developing thromboses affecting 3 or more organs. Emergency treatment is therapeutic anticoagulation, glucocorticoids, plasma exchange and/or IVIG, plus treatment of a trigger. Add cyclophosphamide when associated with active SLE. |
| Diffuse alveolar hemorrhage/pulmonary-renal syndrome | Use the AAV and KDIGO guidance above, with disease-specific anti-GBM or SLE management. | Hemoptysis may be absent. Dyspnea, falling hemoglobin, new diffuse infiltrates, AKI and active urine sediment require ICU-level evaluation. |
High-yield guideline updates
-
AAV: The 2025 BSR guideline and 2024 KDIGO guideline are the current key references for severe renal or pulmonary vasculitis. Plasma exchange is not routine for all AAV, and its use should be individualized, especially in severe renal failure or diffuse alveolar hemorrhage.
-
GCA: Immediate therapy matters more than diagnostic delay. Confirm with ultrasound, biopsy, or other vascular imaging as locally appropriate, but only after treatment has begun if suspicion is high.
-
Systemic sclerosis: ACE inhibition remains the first-line emergency treatment for scleroderma renal crisis. Avoid unnecessary high-dose glucocorticoids because they increase risk.
-
Septic arthritis: Crystal identification does not exclude joint infection. A negative Gram stain also does not exclude it.
-
MAS/HLH: A decline in ESR during ongoing systemic inflammation may be a warning sign because fibrinogen is being consumed.
A current evidence caveat: broad, recent systematic reviews specifically titled “rheumatological emergencies” are scarce. Management is driven mainly by the disease-specific guidelines above rather than a single umbrella guideline.
SBA question set
SBA 1: Hot swollen knee
A 63-year-old woman with rheumatoid arthritis on prednisolone and a biologic agent presents with a painful swollen knee, temperature 37.8°C, and CRP 180 mg/L. Synovial fluid contains monosodium urate crystals. What is the best next step?
A. Diagnose gout and prescribe colchicine
B. Diagnose rheumatoid flare and increase prednisolone
C. Send synovial fluid for Gram stain and culture, obtain blood cultures, and manage as possible septic arthritis
D. Discharge with NSAIDs because crystals explain the symptoms
E. Start allopurinol immediately
B. Diagnose rheumatoid flare and increase prednisolone
C. Send synovial fluid for Gram stain and culture, obtain blood cultures, and manage as possible septic arthritis
D. Discharge with NSAIDs because crystals explain the symptoms
E. Start allopurinol immediately
Answer: C. Crystals do not exclude septic arthritis. Immunosuppression increases infection risk and may blunt fever.
SBA 2: GCA
A 76-year-old woman has a new temporal headache, jaw claudication, ESR 96 mm/hour, and brief episodes of painless monocular visual loss. What is the most appropriate immediate management?
A. Wait for temporal artery biopsy
B. Start oral methotrexate
C. Give immediate high-dose glucocorticoids and arrange urgent ophthalmology/rheumatology review
D. Arrange outpatient MRI brain
E. Give aspirin alone
B. Start oral methotrexate
C. Give immediate high-dose glucocorticoids and arrange urgent ophthalmology/rheumatology review
D. Arrange outpatient MRI brain
E. Give aspirin alone
Answer: C. This is threatened visual loss due to suspected GCA. Treatment must not wait for biopsy.
SBA 3: Pulmonary-renal syndrome
A 42-year-old man has worsening dyspnea, bilateral pulmonary infiltrates, hemoglobin falling from 11.5 to 8.2 g/dL, creatinine 3.0 mg/dL, and urine microscopy showing dysmorphic red cells and red-cell casts. Which diagnosis is most likely?
A. Cardiogenic pulmonary edema
B. Pulmonary embolism
C. Pulmonary-renal syndrome
D. Community-acquired pneumonia
E. Nephrotic syndrome
B. Pulmonary embolism
C. Pulmonary-renal syndrome
D. Community-acquired pneumonia
E. Nephrotic syndrome
Answer: C. Diffuse alveolar hemorrhage with glomerulonephritis is pulmonary-renal syndrome, most often due to AAV, anti-GBM disease, or SLE.
SBA 4: Investigation in pulmonary-renal syndrome
In the preceding patient, which test is particularly important because it may alter urgent management?
A. Rheumatoid factor
B. Anti-GBM antibody
C. Serum urate
D. HLA-B27
E. Anti-CCP antibody
B. Anti-GBM antibody
C. Serum urate
D. HLA-B27
E. Anti-CCP antibody
Answer: B. Anti-GBM disease is an important cause of pulmonary-renal syndrome and commonly requires urgent plasma exchange plus immunosuppression.
SBA 5: Scleroderma renal crisis
A 38-year-old woman with early diffuse systemic sclerosis develops headache, BP 210/120 mmHg, creatinine rise from 0.8 to 2.4 mg/dL, anemia with schistocytes, and thrombocytopenia. What is the most appropriate initial specific treatment?
A. High-dose prednisolone
B. Immediate ACE inhibitor therapy
C. Calcium supplementation
D. Cyclophosphamide alone
E. Stop all antihypertensive medication until renal biopsy
B. Immediate ACE inhibitor therapy
C. Calcium supplementation
D. Cyclophosphamide alone
E. Stop all antihypertensive medication until renal biopsy
Answer: B. This is scleroderma renal crisis. Start an ACE inhibitor urgently.
SBA 6: CAPS
A patient with known APS develops acute kidney injury, livedo reticularis, confusion, thrombocytopenia, and respiratory failure over 4 days following sepsis. Imaging confirms renal and pulmonary thromboses. What is the most likely diagnosis?
A. TTP
B. Disseminated intravascular coagulation only
C. Catastrophic antiphospholipid syndrome
D. Isolated lupus flare
E. Immune thrombocytopenia
B. Disseminated intravascular coagulation only
C. Catastrophic antiphospholipid syndrome
D. Isolated lupus flare
E. Immune thrombocytopenia
Answer: C. Rapid thrombosis involving multiple organs in a patient with APS is CAPS.
SBA 7: CAPS treatment
Which combination best represents first-line treatment for CAPS, in addition to treating the precipitating factor?
A. NSAID + hydroxychloroquine + methotrexate
B. Therapeutic anticoagulation + glucocorticoids + plasma exchange and/or IVIG
C. Aspirin alone
D. Warfarin started without heparin
E. Tocilizumab monotherapy
B. Therapeutic anticoagulation + glucocorticoids + plasma exchange and/or IVIG
C. Aspirin alone
D. Warfarin started without heparin
E. Tocilizumab monotherapy
Answer: B.
SBA 8: MAS
A patient with adult-onset Still disease develops persistent fever, hepatosplenomegaly, falling platelet count, elevated AST, ferritin 38,000 micrograms/L, triglycerides 4.5 mmol/L, and low fibrinogen. What is the diagnosis?
A. Uncomplicated Still disease flare
B. Disseminated gonococcal infection
C. Macrophage activation syndrome
D. Antiphospholipid syndrome
E. Gout
B. Disseminated gonococcal infection
C. Macrophage activation syndrome
D. Antiphospholipid syndrome
E. Gout
Answer: C. The combination of fever, cytopenias, liver dysfunction, hyperferritinemia and hypofibrinogenemia is classic for MAS/secondary HLH.
SBA 9: MAS laboratory clue
Which laboratory trend is particularly concerning for MAS in a patient with systemic JIA or Still disease?
A. Rising fibrinogen
B. Falling ferritin
C. Falling platelet count with rising ferritin and decreasing fibrinogen
D. Isolated positive rheumatoid factor
E. Hypercalcemia
B. Falling ferritin
C. Falling platelet count with rising ferritin and decreasing fibrinogen
D. Isolated positive rheumatoid factor
E. Hypercalcemia
Answer: C.
SBA 10: RA cervical spine
A patient with long-standing rheumatoid arthritis requires emergency intubation after trauma. She has neck pain and hand weakness. Which is the most important precaution?
A. Forceful neck extension for direct laryngoscopy
B. Assume neck pain is due to osteoarthritis
C. Maintain cervical precautions and assess for atlanto-axial instability
D. Perform lumbar puncture before imaging
E. Give intra-articular steroid injection
B. Assume neck pain is due to osteoarthritis
C. Maintain cervical precautions and assess for atlanto-axial instability
D. Perform lumbar puncture before imaging
E. Give intra-articular steroid injection
Answer: C. Atlanto-axial subluxation can result in cord injury during airway manipulation.
SBA 11: SLE and acute dyspnea
A woman with SLE has acute dyspnea, hypoxemia, diffuse bilateral infiltrates, and rapidly falling hemoglobin. There is no overt hemoptysis. What is the most important diagnosis to consider?
A. Pulmonary edema only
B. Diffuse alveolar hemorrhage
C. Panic attack
D. Isolated pleuritis
E. Fibromyalgia
B. Diffuse alveolar hemorrhage
C. Panic attack
D. Isolated pleuritis
E. Fibromyalgia
Answer: B. Hemoptysis can be absent in diffuse alveolar hemorrhage.
SBA 12: General emergency principle
Which statement is most accurate in a patient with established rheumatic disease who develops fever and worsening organ dysfunction?
A. It is usually an autoimmune flare
B. Immunosuppression should always be immediately escalated
C. Infection, thrombosis, drug toxicity, and disease flare must all be assessed urgently
D. ESR alone distinguishes flare from infection
E. Negative blood cultures exclude serious infection
B. Immunosuppression should always be immediately escalated
C. Infection, thrombosis, drug toxicity, and disease flare must all be assessed urgently
D. ESR alone distinguishes flare from infection
E. Negative blood cultures exclude serious infection
Answer: C.
Viva question set with model answers
Viva 1: “List rheumatological emergencies.”
Model answer
I would classify them as:
- Infective: septic arthritis, sepsis or opportunistic infection in an immunosuppressed patient.
- Vascular or thrombotic: giant cell arteritis with visual threat, catastrophic APS, critical digital or mesenteric ischemia.
- Vasculitic organ-threatening disease: pulmonary-renal syndrome, diffuse alveolar hemorrhage, rapidly progressive glomerulonephritis, CNS vasculitis.
- Connective-tissue disease emergencies: scleroderma renal crisis, severe lupus nephritis, neuropsychiatric lupus, alveolar hemorrhage.
- Hyperinflammatory syndromes: MAS/secondary HLH.
- Mechanical or neuromuscular: RA atlanto-axial instability, inflammatory myopathy with bulbar or respiratory weakness.
- Treatment-related: methotrexate toxicity, neutropenic sepsis, serious biologic-associated infection.
Viva 2: “How would you assess a patient with suspected rheumatological emergency?”
Model answer
- Use ABCDE and identify sepsis, shock, hypoxia, encephalopathy, visual loss, AKI, bleeding, and thrombosis.
- Take focused history: rheumatic diagnosis, immunosuppression, recent infection, medication exposure, thrombosis history, pregnancy, and recent procedures.
- Examine for hot joint, rash/purpura, digital ischemia, neurological deficit, lung hemorrhage, hypertension, and ocular signs.
- Request CBC, CRP/ESR, renal/liver profile, urinalysis, coagulation profile, cultures, blood film, ferritin/triglycerides/fibrinogen if MAS is possible, and disease-directed serology.
- Involve ICU and relevant specialists early.
- Do not assume flare until infection and thrombosis are considered.
Viva 3: “How do you manage suspected septic arthritis?”
Model answer
- Regard it as a joint-threatening emergency.
- Assess for sepsis and take blood cultures.
- Aspirate the affected joint urgently for cell count, Gram stain, culture and crystals.
- Begin empiric IV antibiotics after cultures, or immediately if the patient is septic and sampling would delay treatment.
- Obtain urgent orthopedic input for drainage, arthroscopy, arthrotomy, or serial aspiration.
- Tailor antibiotics to microbiological results.
- Remember that crystals and a negative Gram stain do not rule out infection.
Viva 4: “What is your management of suspected GCA with visual symptoms?”
Model answer
- This is a visual emergency.
- Start high-dose glucocorticoids immediately. If there is threatened or established visual loss, use IV pulse glucocorticoids according to local protocol and specialist advice.
- Arrange urgent ophthalmology and rheumatology assessment.
- Send ESR, CRP, CBC and liver tests.
- Arrange temporal artery ultrasound and/or biopsy, but do not delay treatment.
- Assess for large-vessel disease and provide steroid toxicity prevention during ongoing therapy.
Viva 5: “Define pulmonary-renal syndrome and outline management.”
Model answer
Pulmonary-renal syndrome is diffuse alveolar hemorrhage with glomerulonephritis. Key causes are ANCA-associated vasculitis, anti-GBM disease and SLE.
Management includes:
- ICU assessment and respiratory support.
- CBC, renal function, urine microscopy, ANCA, anti-GBM antibodies, complement and lupus serology.
- Chest imaging and consideration of bronchoscopy when appropriate.
- Urgent nephrology and rheumatology input.
- High-dose glucocorticoids and disease-specific induction therapy.
- Anti-GBM disease generally requires plasma exchange in addition to immunosuppression.
- Exclude infection concurrently.
Viva 6: “Describe scleroderma renal crisis.”
Model answer
It is an acute complication of systemic sclerosis characterized by abrupt severe hypertension, AKI, and sometimes microangiopathic hemolytic anemia and thrombocytopenia.
Risk factors include early diffuse cutaneous disease, anti-RNA polymerase III antibodies, rapidly progressive skin disease and glucocorticoid exposure.
The treatment is immediate ACE inhibition, close BP and renal monitoring, ICU/nephrology care where required, and dialysis if needed. An early rise in creatinine is not a reason to stop the ACE inhibitor.
Viva 7: “How would you recognize and treat MAS?”
Model answer
Suspect MAS in a patient with Still disease, systemic JIA, SLE, or another inflammatory disorder who has persistent fever, cytopenias, liver dysfunction, coagulopathy, hypertriglyceridemia, low fibrinogen and markedly elevated ferritin.
I would:
- Stabilize and assess for multiorgan failure.
- Search urgently for infection and malignancy.
- Request serial CBC, LFTs, ferritin, triglycerides, fibrinogen, coagulation profile and hemolysis testing.
- Start high-dose glucocorticoids with urgent rheumatology and hematology input.
- Consider anakinra, cyclosporine, IVIG or other targeted therapy based on cause and severity.
Viva 8: “What is catastrophic APS?”
Model answer
CAPS is a rapidly progressive syndrome of widespread small-vessel thrombosis causing multiorgan failure, usually within a week. It is often triggered by infection, surgery, trauma, pregnancy, anticoagulant withdrawal, malignancy, or active SLE.
Management is ICU-based and includes:
- Therapeutic anticoagulation, usually unfractionated heparin
- High-dose glucocorticoids
- Plasma exchange and/or IVIG
- Identification and treatment of triggers
- Cyclophosphamide if CAPS is associated with active SLE.
Rheumatological Emergencies: One-Page Revision Table
| Emergency | Recognition clues | Key urgent tests | Immediate action | Exam pearl |
|---|---|---|---|---|
| Septic arthritis | Acute hot, swollen, very painful joint with restricted passive movement. Fever may be absent, especially if immunosuppressed. | Urgent joint aspirate: cell count, Gram stain, culture, crystals. Blood cultures. | Manage as sepsis if unwell. Aspirate promptly, then IV antibiotics and urgent orthopedic drainage/washout as indicated. | Crystals do not rule out infection. Negative Gram stain does not exclude it. |
| Giant cell arteritis | Age >50; new headache, scalp tenderness, jaw claudication, visual disturbance or amaurosis fugax, PMR symptoms. | ESR, CRP, CBC, LFTs. Temporal artery ultrasound and/or biopsy. | Give high-dose glucocorticoids immediately. Urgent ophthalmology and rheumatology if visual symptoms. | Do not wait for biopsy or imaging when vision is threatened. |
| Pulmonary-renal syndrome | Dyspnea, hypoxia, diffuse infiltrates, falling Hb, hemoptysis may be absent, AKI with hematuria/RBC casts. | FBC, renal function, urinalysis, ANCA, anti-GBM, ANA/dsDNA, complement, chest imaging. | ICU/respiratory support if needed; urgent renal-rheumatology review; immunosuppression guided by cause after infection assessment. | Think AAV, anti-GBM disease, or SLE. |
| Diffuse alveolar hemorrhage | Acute breathlessness, hypoxia, falling Hb, bilateral infiltrates, with or without hemoptysis. | Serial Hb, CXR/CT, renal screen, autoimmune serology; bronchoscopy if appropriate. | ICU-level care if severe; treat underlying vasculitis/SLE urgently; exclude infection. | Hemoptysis can be absent. |
| ANCA vasculitis with organ threat | Rapid AKI, pulmonary hemorrhage, purpura, neuropathy, scleritis, ischemic gut or digital ischemia. | Urine microscopy, creatinine, ANCA, anti-GBM, complements; biopsy when safe. | High-dose glucocorticoids plus rituximab or cyclophosphamide under specialist care. | Organ-threatening disease needs urgent induction, not outpatient work-up. |
| Scleroderma renal crisis | Systemic sclerosis with abrupt severe hypertension, AKI, headache, heart failure, MAHA/thrombocytopenia. | Frequent BP, creatinine, urinalysis, FBC, blood film, LDH, haptoglobin. | Start ACE inhibitor immediately, commonly rapidly titratable captopril. Involve nephrology. | Do not stop ACE inhibitor solely because creatinine initially rises. |
| Catastrophic APS | Rapid thromboses in 3 or more organs over days: renal, CNS, pulmonary, skin, cardiac, GI. Often triggered by infection. | FBC, coagulation, D-dimer, renal/liver profile, antiphospholipid antibodies; image thromboses. | ICU care; therapeutic heparin + glucocorticoids + plasma exchange and/or IVIG; treat trigger. | Consider cyclophosphamide if associated with active SLE. |
| Macrophage activation syndrome / secondary HLH | Persistent fever, hepatosplenomegaly, cytopenias, hepatitis, coagulopathy, marked hyperferritinemia. | Ferritin, FBC, LFT, triglycerides, fibrinogen, coagulation, infection and malignancy screen. | Urgent rheumatology, hematology and ICU input; high-dose glucocorticoids and targeted immunomodulation. | High ferritin + falling platelets + low fibrinogen is a key pattern. |
| Severe SLE | Lupus nephritis with AKI, pulmonary hemorrhage, seizures/psychosis, myocarditis, severe cytopenias. | Renal profile, urinalysis, complements, anti-dsDNA, hemolysis screen, APS testing, infection screen. | Stabilize affected organ; distinguish flare from infection, APS and drug toxicity; specialist-directed immunosuppression. | In SLE, not every deterioration is a flare. |
| Inflammatory myopathy crisis | Rapid proximal weakness, dysphagia, aspiration, weak cough, respiratory failure, rhabdomyolysis. | CK, electrolytes, creatinine, ABG, FVC/respiratory measures, ECG/troponin if cardiac concern. | Respiratory and swallow assessment, aspiration precautions, ICU if weakness is severe; treat confirmed inflammatory disease. | Dysphagia may be life-threatening due to aspiration. |
| RA cervical spine instability | Long-standing RA with neck/occipital pain, myelopathy, weakness, gait change or symptoms after minor trauma. | Cervical CT/MRI. | Immobilize, urgent spinal review, alert anesthesia before airway intervention. | Avoid forceful neck extension during intubation. |
| Immunosuppression-related infection/toxicity | Fever, hypotension, neutropenia, mucositis, pancytopenia, atypical/opportunistic infection. | Cultures, FBC, renal/liver profile, imaging directed by symptoms. | Sepsis management, antimicrobials, withhold immunosuppression in serious infection unless specialist-directed. | Biologics and steroids can mask fever and inflammatory response. |
Universal emergency rule
ABCDE first. In any deteriorating rheumatology patient, urgently consider:
Infection
Thrombosis
Flare
Drug toxicity
Thrombosis
Flare
Drug toxicity
Useful first-line tests: FBC, CRP/ESR, renal/liver profile, urinalysis, cultures, coagulation profile, and targeted imaging/serology.
Current guidance supports urgent aspiration and source control in suspected septic arthritis, immediate steroid treatment in suspected GCA with visual risk, and immediate ACE inhibition in scleroderma renal crisis. See the SANJO septic arthritis guideline, ACR GCA guidance, and the 2024 BSR systemic sclerosis guideline.Printable one-page PDF:
Rheumatological Emergencies One Page Revision
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Rheumatological Emergency Flashcards
Card 1
Front: What is the immediate priority in a patient with a hot, swollen, painful joint?
Answer: Treat as possible septic arthritis until infection is excluded.
Explanation: Septic arthritis can rapidly destroy cartilage and cause sepsis. Perform urgent joint aspiration for cell count, Gram stain, culture, and crystal analysis. Obtain blood cultures. Crystals or a negative Gram stain do not exclude infection.
Card 2
Front: What samples should be sent from a joint aspirate when septic arthritis is suspected?
Answer:
- Synovial white-cell count and differential
- Gram stain
- Culture and sensitivity
- Crystal analysis
Explanation: These tests help identify infection and distinguish crystal arthritis, but crystal presence does not rule out concurrent infection.
Card 3
Front: In suspected giant cell arteritis with visual symptoms, what should be done before biopsy or imaging?
Answer: Start high-dose glucocorticoids immediately.
Explanation: GCA can cause sudden irreversible blindness. Temporal artery biopsy or ultrasound should be arranged urgently, but treatment must not be delayed if suspicion is high, particularly with amaurosis fugax, diplopia, or visual loss.
Card 4
Front: What triad suggests pulmonary-renal syndrome?
Answer:
- Diffuse alveolar hemorrhage
- Glomerulonephritis
- Acute kidney injury with active urinary sediment
Explanation: Look for dyspnea, hypoxia, falling hemoglobin, diffuse pulmonary infiltrates, hematuria, dysmorphic red cells, or red-cell casts. Key causes are ANCA-associated vasculitis, anti-GBM disease, and SLE.
Card 5
Front: Can hemoptysis be absent in diffuse alveolar hemorrhage?
Answer: Yes.
Explanation: Patients may present only with acute breathlessness, hypoxemia, falling hemoglobin, and new bilateral infiltrates. Absence of hemoptysis does not exclude diffuse alveolar hemorrhage.
Card 6
Front: What is the urgent initial management of organ-threatening ANCA-associated vasculitis?
Answer: High-dose glucocorticoids plus induction immunosuppression, usually rituximab or cyclophosphamide, with urgent specialist involvement.
Explanation: Pulmonary hemorrhage, rapidly progressive glomerulonephritis, critical ischemia, severe neuropathy, or CNS disease are organ-threatening manifestations. Infection must be assessed concurrently.
Card 7
Front: What clinical picture suggests scleroderma renal crisis?
Answer: Systemic sclerosis with abrupt hypertension and acute kidney injury, often with microangiopathic hemolytic anemia and thrombocytopenia.
Explanation: Headache, hypertensive retinopathy, heart failure, encephalopathy, or rapidly rising creatinine may occur. It is particularly associated with early diffuse cutaneous systemic sclerosis.
Card 8
Front: What is the immediate specific treatment for scleroderma renal crisis?
Answer: Start an ACE inhibitor immediately, often captopril because it can be rapidly titrated.
Explanation: ACE inhibition is the key treatment even if creatinine initially rises. Early nephrology involvement and close blood-pressure monitoring are required. High-dose glucocorticoids can increase risk of renal crisis.
Card 9
Front: What is catastrophic antiphospholipid syndrome (CAPS)?
Answer: A rapidly progressive syndrome of widespread thrombosis causing failure of multiple organs over days.
Explanation: It is often triggered by infection, surgery, trauma, pregnancy, anticoagulation withdrawal, malignancy, or active SLE. Renal, neurologic, pulmonary, skin, cardiac, and gastrointestinal involvement may occur.
Card 10
Front: What is the first-line treatment combination for CAPS?
Answer:
- Therapeutic anticoagulation, commonly heparin
- High-dose glucocorticoids
- Plasma exchange and/or IVIG
- Treatment of the precipitating trigger
Explanation: ICU-level multidisciplinary care is often needed. Cyclophosphamide may be added if CAPS occurs with active SLE.
Card 11
Front: What laboratory pattern should raise suspicion of macrophage activation syndrome (MAS)?
Answer: Very high ferritin with falling platelets, cytopenias, elevated transaminases, hypertriglyceridemia, and low fibrinogen.
Explanation: MAS is a hyperinflammatory syndrome, often complicating systemic JIA, adult-onset Still disease, or SLE. Persistent fever, hepatosplenomegaly, coagulopathy, and organ failure may occur.
Card 12
Front: Why can a falling ESR be concerning in suspected MAS?
Answer: It may reflect falling fibrinogen due to consumption, rather than clinical improvement.
Explanation: ESR depends partly on fibrinogen concentration. In MAS, consumption coagulopathy can lower fibrinogen and ESR while the patient is becoming more unwell.
Card 13
Front: What are major severe manifestations of SLE requiring emergency assessment?
Answer:
- Rapidly progressive lupus nephritis
- Diffuse alveolar hemorrhage
- Neuropsychiatric lupus
- Myocarditis
- Severe autoimmune cytopenias
- Thrombotic APS/CAPS
Explanation: Infection, drug toxicity, thrombotic disease, and hypertensive emergency may mimic an SLE flare and must be actively excluded.
Card 14
Front: What features of inflammatory myopathy can make it an emergency?
Answer: Respiratory muscle weakness, severe dysphagia, aspiration, weak cough, rhabdomyolysis, or cardiac involvement.
Explanation: Assess respiratory function, including forced vital capacity where available, arterial blood gases if needed, swallow safety, CK, electrolytes, renal function, and cardiac markers if appropriate.
Card 15
Front: What is the important cervical spine emergency in longstanding rheumatoid arthritis?
Answer: Atlanto-axial subluxation or other cervical spine instability.
Explanation: Suspect it with neck or occipital pain, myelopathic symptoms, gait disturbance, weakness, or neurologic deficits. Immobilize if indicated, arrange urgent CT/MRI, and avoid forceful neck extension during intubation.
Card 16
Front: What are the four major explanations for acute deterioration in a rheumatology patient?
Answer:
- Infection
- Thrombosis
- Flare
- Drug toxicity
Explanation: This “ITFD” framework reduces the risk of wrongly escalating immunosuppression in a patient whose deterioration is due to sepsis, CAPS, medication toxicity, or another non-inflammatory cause.
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