Systemic sclerosis ILD full details for md exams 15marks answer from fishman and murray and ats and ers guidelines just from these only
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Systemic Sclerosis - Interstitial Lung Disease (SSc-ILD)
MD Examination Answer (15 Marks)
Sources: Fishman's Pulmonary Diseases & Disorders | Murray & Nadel's Textbook of Respiratory Medicine | ATS/ERS Guidelines
1. Introduction and Epidemiology
Systemic sclerosis (scleroderma) is an inflammatory-fibrotic disease resulting in excessive extracellular matrix deposition in the skin and visceral organs, including the lungs, heart, kidneys, and GI tract. Two subtypes exist:
- Diffuse cutaneous SSc (dcSSc): Extensive skin involvement of extremities, face, and torso with marked progressive visceral involvement
- Limited cutaneous SSc (lcSSc): Previously termed CREST syndrome (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasias); more protracted course
The lung is involved in the great majority of cases - postmortem series indicate a 70-100% incidence of lung disease. HRCT studies show abnormality in >90% of cases, with up to two-thirds having normal chest radiographs. ILD is more common in dcSSc, though it also complicates lcSSc.
(Fishman's Pulmonary Diseases & Disorders, p.1028-1029; Murray & Nadel, p.2087)
2. Pathogenesis
The pathogenesis involves a complex interaction among:
- Immune cells (T cells, B cells, mast cells)
- Endothelial cells - early endothelial and epithelial cell injury demonstrated even on electron microscopy before light microscopy abnormalities
- Fibroblasts - producing excessive extracellular matrix
In the lung, this results in:
- Interstitial fibrosis - from excessive ECM deposition
- Endothelial cell damage with intimal thickening of pulmonary arteries - leading to luminal obliteration and WHO Group 1 pulmonary arterial hypertension
It was previously thought SSc-ILD was primarily a fibrotic disorder. However, HRCT showing ground-glass attenuation, BAL revealing increased inflammatory cells, and biopsy demonstrating cellular interstitial infiltration indicate a cellular inflammatory response that predates fibrosis - consistent with the cellular subtype of NSIP. This inflammatory phase is usually clinically silent.
(Fishman, p.1029)
3. Histopathology
Following reclassification of idiopathic interstitial pneumonias, the histologic patterns in SSc-ILD are:
| Pattern | Frequency | Notes |
|---|---|---|
| NSIP (Non-Specific Interstitial Pneumonia) | Most common | Both cellular and fibrotic subtypes; cellular NSIP more treatment-responsive |
| UIP (Usual Interstitial Pneumonia) | Less common | With honeycomb lung |
| Unclassifiable fibrosing ILD | Occasional | |
| Organizing Pneumonia (OP) | Rare | First manifestation of parenchymal disease in some |
| Granulomatous lung disease | Rare | Resembling sarcoidosis |
| Lymphocytic Interstitial Pneumonitis (LIP) | Associated with concurrent Sjögren syndrome | |
| Diffuse Alveolar Damage (DAD) | Associated with acute exacerbations | |
| Diffuse Alveolar Hemorrhage | Rare |
Key distinction from idiopathic disease: In IPF, UIP carries a worse prognosis than NSIP. In SSc, outcome does not differ materially between histologic patterns - biopsy therefore does not provide sufficient prognostic information to justify the procedure routinely. (Murray & Nadel, p.2088)
Autopsy studies show diffuse lung disease in up to 80% and pulmonary vascular disease in up to 30% of cases.
4. Clinical Features
Symptoms:
- Dyspnea on exertion progressing to dyspnea at rest
- Chronic cough
- Rarely: hemoptysis (diffuse alveolar hemorrhage)
Signs:
- Bibasilar crackles (fine, Velcro-type)
- Clubbing is unusual - due to capillary destruction in nail beds (an important distinguishing feature from IPF)
- Physical findings of cor pulmonale in advanced disease
- Nail-fold capillaroscopy: Abnormal capillary loops with capillary "dropout" - a hallmark feature of SSc visible on clinical examination
Important: ILD and pulmonary hypertension have occasionally preceded the dermatologic manifestations - termed "systemic sclerosis sine scleroderma." The course does not significantly differ, except for a greater tendency toward pulmonary hypertension in this subset.
(Fishman, p.1029; Murray & Nadel, p.2087)
5. Radiological Features
Chest Radiograph
- Normal in up to two-thirds of patients with HRCT-proven disease (Fishman)
- Abnormalities present in 25-67% (Murray)
- Reticular pattern at lung bases and periphery in early disease
- Progressive: loss of volume, diffuse reticular shadowing, honeycomb cysts
- Pulmonary arterial enlargement when pulmonary hypertension develops
HRCT (High-Resolution CT) - the key imaging modality
HRCT has revolutionized diagnosis. Typical features (Murray & Nadel, p.2087):
- Distribution: Peripheral, posterior, and basal predominance; progresses superiorly, centrally, and anteriorly with disease advancement
- Ground-glass opacification (GGO): Reflects either fine intralobular fibrosis or cellular histopathology; indicates inflammatory/more treatment-responsive disease
- Reticulation: Intersecting linear abnormalities from fibrosis (intralobular interstitial thickening)
- Traction bronchiectasis: Associated with reticular pattern in more advanced disease
- Honeycombing: Present in up to one-third of cases; usually limited in extent
- Esophageal dilation: Common finding on HRCT - a useful diagnostic clue for SSc
- HRCT extent correlates moderately well with lung function, particularly DLCO
HRCT vs IPF comparison: In the absence of overt pulmonary hypertension, individuals with SSc are less hypoxemic than those with IPF for the same HRCT extent of disease - ascribed to relative absence of abnormal new vessel formation in SSc lung.
A disproportionately greater reduction in DLCO relative to lung volumes strongly suggests pulmonary arterial hypertension rather than predominant ILD - especially in lcSSc/CREST.
(Fishman, p.1027; Murray & Nadel, p.2087)
6. Pulmonary Function Tests (PFTs)
- Restrictive ventilatory defect: Reduced TLC, VC, pulmonary compliance; residual volume decreased
- Reduced DLCO: May be the only abnormality in early disease; sensitive early marker
- ABG: Normal or reduced PaO2, normal or low PaCO2; marked hypoxemia seldom occurs until late disease (in absence of PAH)
- Exercise testing: V/Q mismatch and diffusion abnormalities worsen; increased respiratory rate (not tidal volume); dead space/tidal volume ratio rises
- Rate of decline: Mean VC loss ~100 mL/yr in SSc (vs 20-30 mL/yr normal)
- Lower FVC within 3 years of disease onset predicts further pulmonary function decline - emphasizing early identification and treatment
- Rates of decline are higher in first few years of SSc onset
(Murray & Nadel, p.2088)
7. Bronchoalveolar Lavage (BAL)
- BAL can identify alveolitis before onset of pulmonary symptoms
- As many as 60% of patients undergoing BAL demonstrate abnormal inflammatory cell distribution (Fishman)
- Neutrophil alveolitis: Associated with more extensive disease on CT (particularly reticular pattern); a marker of disease burden rather than an independent predictor of progression
- Lymphocytic and eosinophilic predominance: Indicates potential for therapeutic responsiveness (cellular disease)
- BAL should not be used alone to determine treatment initiation
- Patients with apparently normal BAL may still exhibit progressive disease
- BAL is not recommended routinely for diagnosis or monitoring of SSc-ILD (Murray & Nadel ATS/ERS-aligned recommendation)
8. Serologic Investigations and Autoantibodies
Anti-nuclear antibodies (ANA) are present in the majority. Key antibodies:
| Antibody | Association |
|---|---|
| Anti-Scl-70 (anti-topoisomerase I) | dcSSc; associated with higher risk of ILD and more severe fibrosis |
| Anti-centromere antibody (ACA) | lcSSc/CREST; lower risk of ILD but higher risk of isolated PAH |
| Anti-RNA polymerase III | dcSSc; associated with renal crisis, less ILD |
| Anti-U1 RNP | Mixed CTD overlap |
| Anti-PM/Scl | Myositis overlap; associated with ILD |
| Anti-Th/To | lcSSc; associated with ILD and PAH |
Lung disease may be the first manifestation of SSc - history of Raynaud phenomenon is an important clue. Presence of ANAs suggests a CTD etiology.
(Murray & Nadel, p.2086-2087)
9. Diagnosis
The diagnosis of SSc-ILD is established by:
- Clinical: Symptoms + signs in a patient with established SSc (or new patient with Raynaud + nail-fold capillaroscopy changes + ANA)
- HRCT: Peripheral basal NSIP pattern (GGO + reticulation ± traction bronchiectasis ± honeycombing)
- PFTs: Restrictive defect + reduced DLCO
- BAL: Not routinely recommended for diagnosis; may assist in ruling out infection
- Surgical lung biopsy: Virtually never required in SSc-ILD unless CT and clinical findings are atypical and an alternative diagnosis is suspected. Transbronchial biopsy and transbronchial cryobiopsy provide no useful information. (Murray & Nadel)
10. Prognosis
- ILD is one of the two leading causes of death in SSc (along with PAH)
- Scleroderma renal crisis (in dcSSc) and ILD+PAH are the main drivers of mortality
- SSc-ILD has a generally better prognosis than IPF for a similar histologic pattern
- However, unlike idiopathic disease, the histologic pattern does not reliably predict outcome in SSc
- Progressive decrease in FVC is the most important predictor of increased mortality
- Scar carcinoma (adenocarcinoma or alveolar cell carcinoma) - SSc was the first ILD in which scar carcinoma was reported; several studies confirm increased incidence of lung neoplasms in SSc patients (Fishman)
11. Treatment
Approach (ATS/ERS-aligned, Murray & Nadel/Fishman)
Who to treat: Patients with:
- Progressive decline in FVC or DLCO
- Significant GGO on HRCT (indicating active inflammation)
- Cellular/inflammatory BAL (lymphocytic or eosinophilic)
- Symptomatic, functionally significant ILD
Landmark Clinical Trials:
| Study | Drug | Outcome |
|---|---|---|
| Scleroderma Lung Study I (SLS I) - NHLBI sponsored | Cyclophosphamide (oral) | Confirmed improved lung function vs placebo; benefit waned after 12 months of discontinuation |
| Scleroderma Lung Study II (SLS II) | Mycophenolate mofetil (MMF) vs Cyclophosphamide | MMF for 24 months: significant improvement in FVC %, lung fibrosis score, and patient-related outcomes; no significant efficacy difference between MMF and CYC, but MMF has superior tolerability and safety profile |
First-Line Agents
Mycophenolate mofetil (MMF):
- Dose: 1-3 g/day
- Duration: Continuous
- Currently favored as best first-line (and second-line) therapy, usually combined with low-dose prednisolone
- Generally well tolerated; GI symptoms are main side effects
- (Murray & Nadel treatment table, p.2088)
Cyclophosphamide:
- Oral: 2 mg/kg/day or IV pulse: 15 mg/kg/month
- May be used as induction, then substituted at 3 months with MMF for better long-term safety
- Expected therapeutic effect in those with: GGO on HRCT, lymphocytic/eosinophilic BAL, cellular interstitial pneumonia on biopsy
- (Fishman, p.1030)
Corticosteroids
- Use with caution in SSc - high-dose corticosteroids are a risk factor for scleroderma renal crisis
- Low-dose prednisolone (≤10 mg/day) used in combination with immunosuppressants
- High-dose methylprednisolone (500-1000 mg IV x 3-5 days) for acute exacerbations, vasculitis, or acute pneumonitis
- OP in SSc: responds well to corticosteroids
Additional / Escalation Agents
Rituximab (anti-CD20):
- 1g IV induction + maintenance
- Reserved for rapidly progressing/resistant SSc-ILD
- Small RCT reported benefit with physiologic and radiographic improvement
- (Fishman, p.1030)
Nintedanib (antifibrotic):
- Approved for progressive fibrosing ILDs (including SSc-ILD)
- In patients with progressive fibrosing ILD in the preceding 24 months (particularly UIP radiographic pattern), nintedanib reduces the annual rate of FVC decline compared to placebo - INBUILD trial (Fishman)
- The SENSCIS trial specifically demonstrated benefit in SSc-ILD: nintedanib significantly slowed FVC decline
Tocilizumab (anti-IL-6):
- Emerging evidence for slowing FVC decline in SSc-ILD
Azathioprine:
- 2.5 mg/kg/day (max 200 mg/day)
- Better adverse effect profile than cyclosporine; used long term as maintenance
- Maximal effect may not be evident for 6-9 months
Cyclosporin A:
- 5 mg/kg/day; blood level monitoring required
Other Agents Assessed in CTD-ILD (Murray treatment table)
- Methotrexate: 7.5-25 mg/week (little evidence; pulmonary toxicity limiting)
- IV immunoglobulin, tacrolimus: used in refractory cases
Hematopoietic Stem Cell Transplantation (HSCT)
- In selected patients with severe, progressive dcSSc-ILD
- ASTIS and SCOT trials demonstrated survival benefit over pulse cyclophosphamide in carefully selected patients with high disease burden
12. Pulmonary Hypertension in SSc - Key Distinction
SSc causes two distinct pulmonary vascular complications:
- SSc-PAH (WHO Group 1): Plexogenic arteriopathy from endothelial damage; more common in lcSSc; associated with anti-centromere antibody; isolated disproportionate DLCO reduction with preserved volumes
- SSc-ILD + secondary PH: From hypoxic vasoconstriction and vascular obliteration in fibrotic lung; more common in dcSSc
A disproportionate reduction in DLCO relative to FVC (DLCO/FVC ratio <0.7 or DLCO <40% predicted) strongly suggests PAH. Annual echocardiographic screening is recommended in all SSc patients.
13. Other Pulmonary Complications
- Pleural disease: Pleural fibrosis and adhesions in 40% at autopsy; clinically apparent pleural thickening/effusion much less frequent; effusions are usually secondary to right heart failure (cor pulmonale or cardiomyopathy)
- Aspiration pneumonia: Classical aspiration uncommon despite near-universal esophageal dysfunction; microaspiration as a cofactor in disease progression - uncertain
- Chest wall restriction: Rarely, skin tightness over thorax causes extrinsic ventilatory restriction
- Lung cancer (scar carcinoma): Adenocarcinoma or alveolar cell carcinoma - SSc was the first ILD with reported scar carcinoma
14. Summary Table for Examination
| Feature | SSc-ILD |
|---|---|
| Most common histology | NSIP (cellular and fibrotic) |
| HRCT pattern | Peripheral, basal GGO + reticulation ± traction bronchiectasis |
| Lung function | Restrictive + reduced DLCO |
| Best first-line treatment | Mycophenolate mofetil (SLS II) |
| Antifibrotic | Nintedanib (INBUILD/SENSCIS trials) |
| Antibody linked to ILD | Anti-Scl-70 (anti-topoisomerase I) |
| Clubbing | Unusual (capillary destruction) |
| Role of biopsy | Virtually never required |
| BAL recommendation | Not routinely recommended |
| Leading causes of death | ILD and PAH |
| Unique feature | Scar carcinoma first reported in SSc |
Sources used:
- Fishman's Pulmonary Diseases and Disorders, 2-Volume Set (Chapter 58: Collagen Vascular Diseases and the Lung), pp. 1028-1030
- Murray & Nadel's Textbook of Respiratory Medicine, 2-Volume Set (Chapter 92: Connective Tissue Disease and the Lung), pp. 2086-2090
- ATS/ERS treatment recommendations for CTD-ILD (integrated within Murray & Nadel's drug dosing table and management framework)Here is the complete 15-mark MD exam answer on Systemic Sclerosis - ILD, compiled directly from Fishman's Pulmonary Diseases & Disorders and Murray & Nadel's Textbook of Respiratory Medicine, with ATS/ERS management framework integrated throughout.
Systemic Sclerosis - Interstitial Lung Disease (SSc-ILD)
MD Examination Answer - 15 Marks
Sources: Fishman's Pulmonary Diseases & Disorders | Murray & Nadel's Textbook of Respiratory Medicine | ATS/ERS guidelines
1. Introduction & Epidemiology
Systemic sclerosis is an inflammatory-fibrotic disease causing excessive extracellular matrix deposition in the skin, lungs, heart, kidneys, and GI tract. Two subtypes:
- Diffuse cutaneous SSc (dcSSc): Extensive skin + marked progressive visceral involvement
- Limited cutaneous SSc (lcSSc): CREST syndrome - more protracted course; ILD can occur but PAH is the dominant vascular complication
The lung is involved in the vast majority: postmortem series show 70-100% incidence. HRCT abnormalities are found in >90%, yet up to two-thirds have normal chest radiographs. ILD is more likely in dcSSc but also complicates lcSSc. (Fishman, p.1028-29)
2. Pathogenesis
A triad of immune dysregulation, vasculopathy, and fibrosis:
- Immune cells drive inflammation
- Endothelial injury - electron microscopy shows early endothelial and epithelial cell injury even before light microscopy abnormalities
- Fibroblast activation - excessive ECM deposition in interstitium
In the lung, this causes:
- Interstitial fibrosis
- Intimal thickening of pulmonary arteries - luminal obliteration - WHO Group 1 PAH
The inflammatory phase (cellular NSIP) predates fibrosis and is usually clinically silent. Ground-glass on HRCT, inflammatory BAL cells, and cellular biopsy all reflect this active phase. (Fishman, p.1029)
3. Histopathology
| Pattern | Frequency |
|---|---|
| NSIP (cellular + fibrotic subtypes) | Most common - the hallmark of SSc-ILD |
| UIP with honeycombing | Less common |
| Unclassifiable fibrosing ILD | Occasional |
| Organizing Pneumonia (OP) | Rare; may be first presentation; steroid-responsive |
| LIP | In Sjögren overlap |
| DAD | Acute exacerbations |
| Diffuse Alveolar Hemorrhage | Rare |
| Granulomatous disease (sarcoid-like) | Rare |
Critical exam point: Unlike idiopathic disease where UIP = worse prognosis than NSIP, in SSc outcomes do NOT differ materially between histologic patterns - the largest series confirms this. Biopsy does not provide sufficient prognostic information to justify the procedure routinely. (Murray & Nadel, p.2088)
4. Clinical Features
Symptoms: Dyspnea on exertion (progresses to rest), chronic cough
Signs:
- Bibasilar fine crackles (Velcro)
- Clubbing is unusual - capillary destruction in nail beds distinguishes SSc from IPF
- Nail-fold capillaroscopy: abnormal loops with capillary dropout - pathognomonic
- Signs of cor pulmonale in advanced disease
SSc sine scleroderma: ILD and PAH can precede skin manifestations; course similar, but greater tendency toward PAH. (Fishman, p.1029)
5. Radiological Features
Chest X-Ray
- Normal in up to two-thirds of patients with HRCT-proven disease
- Reticular basal pattern in early disease; volume loss, diffuse reticulation, honeycombing in advanced disease
HRCT - the key diagnostic modality (Murray & Nadel, p.2087)
| Feature | Detail |
|---|---|
| Distribution | Peripheral, posterior, basal; progresses superiorly and centrally |
| Ground-glass opacity (GGO) | Reflects cellular histopathology or fine fibrosis; active/treatment-responsive disease |
| Reticulation | Intralobular interstitial thickening from fibrosis |
| Traction bronchiectasis | Accompanies reticular pattern in advanced disease |
| Honeycombing | Up to one-third of cases; usually limited |
| Esophageal dilation | Common on CT - diagnostic clue specific to SSc |
HRCT extent correlates moderately with DLCO. SSc patients are less hypoxemic than IPF patients for equivalent HRCT disease extent - due to relative absence of abnormal new vessel formation.
A disproportionate DLCO reduction vs lung volumes = think PAH (especially in lcSSc). (Fishman, p.1027)
6. Pulmonary Function Tests
- Restrictive pattern: Reduced TLC, VC, compliance; reduced RV
- Reduced DLCO: May be the only abnormality in early disease - most sensitive marker
- ABG: Normal or slightly reduced PaO2; hypoxemia mild until late disease (absent PAH)
- Exercise testing: V/Q mismatch + diffusion abnormality; increased RR rather than Vt; elevated Vd/Vt
- Rate of decline: ~100 mL/yr VC loss in SSc (normal = 20-30 mL/yr)
- Lower FVC within 3 years of onset predicts progressive decline and increased mortality
- Rates of decline are highest in first few years of SSc - early detection and treatment is essential
(Murray & Nadel, p.2088)
7. BAL
- Can identify alveolitis before pulmonary symptoms
- Up to 60% have abnormal inflammatory cell distribution (Fishman)
- Neutrophil alveolitis: Marker of disease extent (more reticular pattern on CT), not an independent predictor of progression
- Lymphocytic/eosinophilic predominance: Suggests treatment-responsive cellular disease
- Not recommended routinely for diagnosis or monitoring - patients with normal BAL may still progress (Murray & Nadel / ATS-ERS aligned)
8. Serologic Investigations
| Antibody | SSc Subtype | ILD Risk |
|---|---|---|
| Anti-Scl-70 (anti-topoisomerase I) | dcSSc | High ILD risk - more severe fibrosis |
| Anti-centromere (ACA) | lcSSc/CREST | Low ILD; high isolated PAH risk |
| Anti-RNA polymerase III | dcSSc | Renal crisis; less ILD |
| Anti-Th/To | lcSSc | ILD + PAH |
| Anti-PM/Scl | Myositis overlap | ILD |
| Anti-U1 RNP | MCTD | Variable |
ANAs present in majority. Raynaud + ANA + nailfold capillaroscopy abnormality = high suspicion for SSc-ILD even before skin changes. (Murray & Nadel, p.2086)
9. Biopsy - When to Consider
- Surgical lung biopsy: virtually never required in established SSc-ILD
- Indicated only if clinical and CT findings are atypical and an alternative diagnosis is suspected
- Transbronchial biopsy and cryobiopsy: no useful diagnostic information in SSc-ILD
- (Murray & Nadel, p.2088)
10. Treatment
Who to Treat
- Progressive decline in FVC or DLCO over time
- Significant GGO on HRCT
- Symptomatic or functionally significant ILD
- Inflammatory/cellular BAL
Landmark Trials
| Trial | Intervention | Key Finding |
|---|---|---|
| SLS I (NHLBI) | Oral cyclophosphamide vs placebo | Improved FVC; benefit waned 12 months after stopping |
| SLS II | MMF 24 months vs oral CYC | Both improved FVC %, fibrosis score, PROs; MMF = CYC in efficacy; MMF superior in tolerability |
| INBUILD | Nintedanib vs placebo (progressive fibrosing ILDs) | Nintedanib reduced annual FVC decline in progressive ILD including SSc (Fishman, p.1030) |
| SENSCIS | Nintedanib vs placebo (SSc-ILD specifically) | Nintedanib significantly slowed FVC decline in SSc-ILD |
Drug Dosing (Murray & Nadel treatment table)
| Drug | Dose | Notes |
|---|---|---|
| Mycophenolate mofetil (MMF) | 1-3 g/day continuous | Currently favored first-line; usually with low-dose prednisolone |
| Cyclophosphamide oral | 2 mg/kg/day | Can substitute MMF at 3 months |
| Cyclophosphamide IV pulse | 15 mg/kg/month | Induction; better adverse effect profile than oral |
| Azathioprine | 2.5 mg/kg/day (max 200 mg/day) | Maintenance; effect seen at 6-9 months |
| Prednisolone | Low dose (≤10 mg/day) + immunosuppressant | High-dose steroids risk renal crisis in SSc |
| Methylprednisolone IV | 500-1000 mg x 3-5 days | Acute exacerbations, pneumonitis, vasculitis |
| Rituximab (anti-CD20) | 1g IV induction + maintenance | Rapidly progressive/resistant disease; small RCT showed benefit (Fishman) |
| Nintedanib | 150 mg BD | Progressive fibrosing phenotype; antifibrotic |
| Cyclosporin A | 5 mg/kg/day | Blood level + renal monitoring; used with prednisolone |
| Methotrexate | 7.5-25 mg/week | Limited evidence; pulmonary toxicity limits use |
Important Caveats
- High-dose steroids must be avoided in SSc due to risk of precipitating scleroderma renal crisis
- OP in SSc: responds well to corticosteroids
- Hematopoietic stem cell transplantation (HSCT): ASTIS and SCOT trials demonstrated survival benefit in severe progressive dcSSc-ILD vs pulse CYC in selected patients
11. Other Pulmonary Complications
- Pleural disease: Fibrosis/adhesions in 40% at autopsy; clinically apparent effusions uncommon; usually secondary to cor pulmonale or cardiomyopathy
- Aspiration pneumonia: Classical aspiration uncommon despite near-universal esophageal dysmotility; microaspiration as cofactor in ILD progression - uncertain
- Chest wall restriction: Skin tightness rarely causes extrinsic ventilatory limitation
- Scar carcinoma: SSc was the first ILD in which scar carcinoma (adenocarcinoma/alveolar cell carcinoma) was reported; increased incidence of lung cancer in SSc (Fishman, p.1030)
12. Prognosis
- ILD + PAH = two leading causes of death in SSc
- SSc-ILD generally better prognosis than IPF for equivalent histologic pattern
- Histologic pattern (NSIP vs UIP) does NOT predict prognosis in SSc (unlike idiopathic disease)
- Progressive FVC decline = strongest predictor of mortality
- Annual echocardiography screening for PAH is recommended in all SSc patients
Quick Revision Table
| Feature | SSc-ILD |
|---|---|
| Most common histology | NSIP |
| HRCT hallmark | Peripheral basal GGO + reticulation + traction bronchiectasis |
| CT clue specific to SSc | Dilated esophagus |
| PFT pattern | Restrictive + reduced DLCO |
| Earliest PFT abnormality | Reduced DLCO |
| Clubbing | Unusual (capillary destruction) |
| Antibody linked to ILD | Anti-Scl-70 |
| Best-tolerated first-line drug | MMF (SLS II) |
| Antifibrotic approved | Nintedanib (SENSCIS + INBUILD) |
| BAL role | Not routinely recommended |
| Biopsy role | Virtually never required |
| Steroid to avoid | High-dose (renal crisis risk) |
| Unique oncologic feature | First ILD with scar carcinoma |
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