Orris

I have to present a case on posterior uveitis, explain every causes and give me short summery for them, viva question to expect with answers, and lastly give me brief management protocol in systematic manner.

Reading File
Finding Sources
Finding Sources
Finding Sources
Reading File
Reading File
Finding Sources
Finding Sources
Reading File
Reading File
Finding Sources
Finding Sources
Reading File
Reading File
Finding Sources
Reading File
Reading File
Reading File
Reading File
I now have a solid, comprehensive evidence base from Kanski's 10th edition and Wills Eye Manual. Let me compile the full case presentation.

Posterior Uveitis - Complete Case Presentation Guide

Sources: Kanski's Clinical Ophthalmology 10th Ed. | Wills Eye Manual | Firestein's Rheumatology | Harrison's Principles

DEFINITION & CLASSIFICATION

Posterior uveitis encompasses retinitis, choroiditis, and retinal vasculitis with the primary site of inflammation being posterior to the vitreous base. It is classified by the SUN (Standardization of Uveitis Nomenclature) Working Group as:
  • Isolated retinitis (white lesions - retinal origin)
  • Isolated choroiditis (yellow lesions - choroidal origin)
  • Retinochoroiditis - primary retinal with secondary choroidal involvement
  • Chorioretinitis - primary choroidal with secondary retinal involvement
  • Retinal vasculitis - venous, arterial, or mixed
  • Neuroretinitis - optic disc + macular star
Key Clinical Features (Kanski's):
  • Active retinitis: white opacities with indistinct (hazy) borders due to surrounding oedema
  • Active choroiditis: round yellow nodule, minimal vitritis unless retina involved
  • Active vasculitis: yellowish/grey-white perivascular cuffing +/- hemorrhage
  • Floaters (peripheral lesion) vs. central visual loss (macular lesion)

CAUSES OF POSTERIOR UVEITIS

GROUP 1: INFECTIOUS CAUSES

1. Toxoplasmosis (Most Common Cause Worldwide)

Organism: Toxoplasma gondii (obligate intracellular protozoan) Mechanism: Reactivation of dormant bradyzoite cysts in the retina (congenital) or primary acquired infection. Immune reactivation triggers focal necrotizing retinitis. Classic appearance: "Headlight in the fog" - active white/yellow focal necrotizing retinal lesion adjacent to an old pigmented chorioretinal scar. Location: Usually near the disc or macula (posterior pole predilection). Vitritis: Moderate to severe overlying the lesion. Associations: AIDS (atypical - multifocal, bilateral, without scar); immunocompetent patients (unilateral, focal). Treatment: Pyrimethamine + Sulfadiazine + Folinic acid ("classic triple therapy") OR Trimethoprim-Sulfamethoxazole. Add systemic steroids (prednisone) 24-48 hrs after starting antimicrobials if vision-threatening. Clindamycin is an alternative.

2. Cytomegalovirus (CMV) Retinitis

Organism: CMV (herpesvirus) Mechanism: Opportunistic infection in immunocompromised hosts (CD4 <50 cells/μL in HIV). Classic appearance: "Pizza pie" or "cottage cheese and ketchup" - confluent areas of retinal necrosis with hemorrhage and white granular opacities. Starts peripherally, spreads centripetally. Key features: Painless, no anterior segment reaction, minimal vitritis (unlike toxoplasmosis). Treatment: IV/oral Ganciclovir (or Valganciclovir), Foscarnet, or Cidofovir. Intravitreal ganciclovir implant for severe cases. Immune recovery uveitis (IRU) can occur after HAART initiation.

3. Acute Retinal Necrosis (ARN)

Organism: VZV (most common), HSV-1, HSV-2 Mechanism: Herpetic necrotizing retinitis via viral replication in the retinal pigment epithelium and neural retina; often bilateral if untreated. Classic triad:
  1. Peripheral necrotizing retinitis (starts peripherally, spreads circumferentially)
  2. Occlusive arteritis
  3. Moderate-severe vitritis Risk: Rhegmatogenous retinal detachment in 50-75% of untreated cases. Treatment: IV Acyclovir (10 mg/kg TID for 7-10 days) followed by oral Valacyclovir (long term). Laser prophylaxis to prevent retinal detachment. Vitrectomy if RD occurs.

4. Progressive Outer Retinal Necrosis (PORN)

Organism: VZV predominantly Mechanism: Seen in severely immunocompromised (AIDS CD4 <50). Multifocal outer retinal necrosis, rapidly progressive, minimal inflammation. Key features: Minimal vitritis, no vascular sheathing (unlike ARN), rapid progression to total retinal necrosis. Treatment: IV Ganciclovir + Foscarnet (combination). Poor prognosis.

5. Ocular Tuberculosis

Organism: Mycobacterium tuberculosis Mechanism: Haematogenous seeding to the choroid; hypersensitivity reaction to mycobacterial antigens. Appearances: Choroidal tubercle (single or multifocal), serpiginous-like choroiditis, subretinal abscess (rare), retinal vasculitis (periphlebitis - "candle wax drippings"), panuveitis. Key investigations: IGRA (QuantiFERON-TB Gold), Mantoux test, CXR/CT chest, PCR of aqueous/vitreous. Treatment: Anti-TB therapy (4-drug HRZE regimen for 6-9 months) + adjunctive systemic steroids for inflammation control.

6. Ocular Syphilis

Organism: Treponema pallidum Mechanism: Secondary or tertiary syphilis; direct spirochetal invasion of the eye; can mimic virtually any form of uveitis ("The Great Masquerader"). Appearances: Granulomatous uveitis, retinitis, chorioretinitis, exudative retinal detachment, papillitis, retinal vasculitis. Key warning: Must be excluded in ALL cases of uveitis. Rising incidence including ocular syphilis with HIV co-infection. Investigations: RPR/VDRL (non-treponemal) + FTA-ABS or TPHA (treponemal), HIV test. Treatment: IV Penicillin G (18-24 million units/day for 14 days) as for neurosyphilis. Oral doxycycline if penicillin allergic.

7. Ocular Toxocariasis

Organism: Toxocara canis or T. cati (larva migrans) Mechanism: Larval migration into the posterior segment triggering intense eosinophilic granulomatous reaction. Appearances: Three forms - (a) posterior pole granuloma (near disc/macula), (b) peripheral granuloma + vitreous traction bands, (c) chronic endophthalmitis-like picture. Epidemiology: Children (age 2-10), history of pica or contact with dogs. Investigations: Toxocara ELISA (serum). ANCA and eosinophilia may be elevated. Treatment: Anti-helminthics (Albendazole/Mebendazole) of uncertain benefit. Systemic corticosteroids to control inflammation. Vitrectomy for vitreous bands/traction.

8. Fungal Endophthalmitis (Candida, Aspergillus)

Organism: Candida albicans (most common), Aspergillus, Cryptococcus, Coccidioides Mechanism: Hematogenous seeding (Candida) in IV drug users, immunocompromised, or post-surgical; direct inoculation (Aspergillus). Classic appearance (Candida): White-yellow fluffy "string of pearls" chorioretinal lesions +/- vitritis. Can evolve to endophthalmitis. Treatment: Intravitreal Voriconazole or Amphotericin B + systemic antifungals. Vitrectomy for extensive disease.

9. Cat-Scratch Disease (Bartonella)

Organism: Bartonella henselae Classic appearance: Unilateral stellate macular exudates, optic nerve swelling (neuroretinitis), vitreous cells; positive Bartonella serology. History: Cat scratch or bite. Treatment: Azithromycin or Doxycycline. Usually self-limiting.

10. Diffuse Unilateral Subacute Neuroretinitis (DUSN)

Organism: Nematode (Toxocara, Baylisascaris, Ancylostoma) Mechanism: Subretinal migration of a live worm; toxin-mediated retinal damage. Appearances: Unilateral - early: optic disc swelling + focal grey-white outer retinal lesions; late: optic atrophy, RPE atrophy, vascular attenuation. Treatment: Laser photocoagulation of visible nematode (definitive). Albendazole if worm not visible.

11. Lyme Disease

Organism: Borrelia burgdorferi Mechanism: Multisystem inflammatory disease; varied ocular manifestations. Appearances: Intermediate uveitis (most common ocular finding), posterior uveitis, retinal vasculitis, papillitis, exudative retinal detachment, panuveitis. Epidemiology: Tick-bite history, travel to endemic areas. Treatment: IV Ceftriaxone for ocular involvement + systemic steroids adjunctively.

GROUP 2: INFLAMMATORY / AUTOIMMUNE CAUSES

12. Sarcoidosis

Mechanism: Systemic non-caseating granulomatous disease; can affect any part of the uveal tract; most common cause of granulomatous uveitis overall. Posterior features: Choroidal granulomas, retinal vasculitis (periphlebitis with "candle wax drippings"), snowball/snowbank vitreous opacities, panuveitis, optic disc granuloma. Systemic features: Hilar lymphadenopathy, erythema nodosum, lupus pernio, pulmonary infiltrates, elevated serum ACE, hypercalcaemia, elevated lysozyme. Investigations: Serum ACE + lysozyme, CXR/HRCT chest, gallium scan, tissue biopsy (conjunctival, lacrimal, bronchoscopy), Kveim test (historical). Treatment: Systemic prednisolone (first-line), steroid-sparing immunosuppressants (Methotrexate, Mycophenolate), Anti-TNF agents (Infliximab, Adalimumab) for refractory cases.

13. Vogt-Koyanagi-Harada (VKH) Syndrome

Mechanism: T-cell mediated autoimmune attack against melanocytes in the uvea, inner ear, meninges, and skin. Posterior features: Bilateral exudative retinal detachments (multi-lobular, serous), choroidal thickening, disc hyperaemia; later: "sunset glow fundus," Dalen-Fuchs nodules. Systemic features: Prodrome of headache, tinnitus, meningismus; later vitiligo, poliosis, alopecia, dysacusis. Investigations: LP (lymphocytic pleocytosis), MRI brain, fluorescein angiography (multiple subretinal leakage points), OCT (subretinal fluid), HLA-DR4/DRw53 association. Treatment: High-dose systemic corticosteroids early and long-term tapering; immunosuppressants (Azathioprine, Mycophenolate) as steroid-sparing agents.

14. Sympathetic Ophthalmia

Mechanism: Bilateral granulomatous panuveitis triggered by sensitization to uveal antigens after penetrating trauma or intraocular surgery to one eye (exciting eye). Classic feature: Fellow (sympathizing) eye develops uveitis within 2 weeks to years after injury. Histology: Dalen-Fuchs nodules (between RPE and Bruch's membrane). Treatment: Evisceration of exciting eye (within 2 weeks of injury, before sympathetic eye inflammation) may prevent. Systemic high-dose corticosteroids + immunosuppressants (Cyclosporin, Azathioprine) for established disease.

15. Behcet Disease

Mechanism: Systemic vasculitis of unknown etiology; HLA-B51 association; immune complex-mediated occlusive vasculitis. Posterior features: Retinal vasculitis (BOTH arteries and veins affected - characteristic), focal necrotizing retinitis, branch/central retinal artery or vein occlusion, disc oedema, hypopyon (shifts - "shifting hypopyon"). Systemic features (triad): Oral ulcers (most common), genital ulcers, uveitis. Pathergy test positive. Prognosis: Poorest visual prognosis of all uveitic conditions if untreated; blindness in up to 25% within 5 years. Treatment: Colchicine (for recurrence prevention), systemic corticosteroids, Azathioprine, Cyclosporin. Anti-TNF agents (Infliximab, Adalimumab) are highly effective for ocular Behcet. Interferon-alpha for refractory cases.

16. Birdshot Chorioretinopathy (Vitiliginous Chorioretinitis)

Mechanism: Autoimmune, strongly associated with HLA-A29 (>95% of patients - strongest HLA association in uveitis). Classic appearance: Bilateral, multiple, small, cream-colored depigmented choroidal lesions scattered around the disc and posterior pole (like birdshot pellets), retinal vasculitis, vitreous cells, CME, disc swelling. Patients: Middle-aged Caucasian females predominantly. Investigations: HLA-A29 testing (highly specific), ERG (reduced amplitude - key for monitoring), fluorescein and ICG angiography, OCT. Treatment: Systemic corticosteroids, immunosuppressants (Mycophenolate, Cyclosporin, Methotrexate). Anti-TNF agents for refractory cases. Long-term treatment required.

17. White Dot Syndromes (Brief Overview)

SyndromeAgeLateralityKey Feature
APMPPEYoung adultsBilateralMultifocal placoid yellow-white lesions; post-viral; self-limiting
Serpiginous ChoroiditisMiddle-agedBilateralGeographic, centrifugal spread from disc; "snail-track" scarring
MEWDSYoung womenUnilateralMultiple evanescent white dots; post-viral; excellent prognosis
PIC (Punctate Inner Choroidopathy)Young myopic womenBilateralSmall yellow punctate lesions; choroidal neovascularization risk
MFC (Multifocal Choroiditis + Panuveitis)Young womenBilateral"Punched-out" lesions mimicking histoplasmosis + vitritis
Subretinal Fibrosis + UveitisYoung womenBilateralProgressive subretinal fibrosis; poor prognosis

18. Primary Intraocular Lymphoma (PIOL) - Masquerade Syndrome

Mechanism: Large B-cell lymphoma of vitreoretinal origin; can masquerade as posterior uveitis (hence "masquerade syndrome"). Classic features: Elderly patients (>50 years), steroid-resistant vitritis, sub-RPE infiltrates (creamy-yellow), "leopard skin" RPE changes. Always consider CNS lymphoma (50% have concurrent CNS involvement). Red flags: Vitritis not responding to steroids in older patients. Investigations: Vitrectomy with undiluted vitreous cytology + IL-10:IL-6 ratio >1, MYD88 mutation. MRI brain/spine. Treatment: Intravitreal Methotrexate and/or Rituximab; systemic chemotherapy + CNS directed therapy.

19. Multiple Sclerosis

Mechanism: Autoimmune demyelinating disease; pars planitis/intermediate uveitis most common, but can cause retinal vasculitis/periphlebitis. Ocular features: Intermediate > posterior uveitis, periphlebitis, CME, optic neuritis (not uveitis per se). Treatment: Systemic steroids for acute flares; DMT for MS.

20. Systemic Lupus Erythematosus (SLE)

Mechanism: Immune complex deposition causing retinal vasculitis (lupus retinopathy), cotton wool spots, vaso-occlusion. Posterior features: Retinal vasculitis (both arteries and veins), vascular occlusions, CME, haemorrhages. Treatment: Treat underlying SLE (hydroxychloroquine, immunosuppressants).

GROUP 3: RARE / MISCELLANEOUS

  • Eales Disease: Young South Asian males; idiopathic retinal periphlebitis (venous sheathing peripherally), neovascularization, vitreous hemorrhage. Treat with laser + steroids.
  • IRVAN (Idiopathic Retinal Vasculitis, Aneurysms, Neuroretinitis): Young females; macroaneurysms on mid-peripheral arteries, neuroretinitis, retinal vasculitis.
  • Frosted Branch Angiitis: Extensive bilateral perivascular exudates covering vessels like ice crystals; associated with CMV, EBV, HSV; treat underlying cause.
  • Whipple Disease: Tropheryma whipplei; oculomasticatory myorhythmia (pathognomonic), posterior uveitis/vitritis. Treat with TMP-SMX.
  • Onchocerciasis ("River Blindness"): Onchocerca volvulus; RPE atrophy, sclerosing choroiditis. Endemic in sub-Saharan Africa. Treat with Ivermectin.

VIVA QUESTIONS AND ANSWERS

Q1. Define posterior uveitis and classify it anatomically. A: Posterior uveitis is inflammation of the uveal tract posterior to the vitreous base. Per SUN classification, it includes isolated retinitis, choroiditis, retinochoroiditis, chorioretinitis, retinal vasculitis, and neuroretinitis. Anterior chamber cells are absent or minimal; vitreous cells may be present as spill-over.
Q2. What is the most common cause of posterior uveitis worldwide? A: Toxoplasmosis is the most common cause of posterior uveitis globally. It presents classically as "headlight in the fog" - a focal necrotizing retinal lesion adjacent to an old pigmented chorioretinal scar.
Q3. What is the pathognomonic sign of toxoplasmosis retinitis? A: "Headlight in the fog" - an active, white fluffy lesion adjacent to (or at the border of) an old pigmented chorioretinal scar, with overlying vitritis causing the "fog." This is because most cases are reactivation of congenital cysts.
Q4. How do you differentiate ARN from CMV retinitis? A:
FeatureARNCMV Retinitis
Immune statusOften immunocompetentSeverely immunocompromised (CD4 <50)
OrganismVZV/HSVCMV
LocationPeripheral, spreads posteriorlyAny location; starts at equator
VitritisProminentMinimal
Vascular occlusionOcclusive arteritisHemorrhagic pattern
Anterior inflammationPresentAbsent
Bilateral30-70% if untreatedLess common
Q5. What is "sunset glow fundus" and in which condition is it seen? A: Sunset glow fundus is a diffuse orange-red depigmentation of the fundus seen in the chronic/convalescent phase of VKH syndrome. It results from destruction of uveal and RPE melanocytes, giving the choroidal vessels a prominent red-orange background hue. It also occurs in sympathetic ophthalmia.
Q6. What is the strongest HLA association in uveitis? A: HLA-A29 with Birdshot Chorioretinopathy (present in >95% of patients). This is the strongest HLA-disease association in all of uveitis. Other important ones: HLA-B27 (anterior uveitis/AS), HLA-B51 (Behcet disease), HLA-DR4/DRw53 (VKH syndrome).
Q7. What is a Dalen-Fuchs nodule? Where is it seen? A: Dalen-Fuchs nodules are aggregates of epithelioid cells between the RPE and Bruch's membrane, representing choroidal granulomas. They appear as small, yellow-white discrete lesions scattered across the fundus. Seen in: VKH syndrome and Sympathetic Ophthalmia (pathognomonic of the latter histologically).
Q8. What is the "shifting hypopyon" and in which condition does it occur? A: Shifting hypopyon is a hypopyon that changes position/side with body movement (due to low fibrin content and high fluidity). It is characteristic of Behcet disease. In contrast, the fibrinous hypopyon of HLA-B27 uveitis is fixed. Importantly, patients with Behcet disease almost never have posterior synechiae despite a severe hypopyon.
Q9. What investigations would you order for a case of posterior uveitis? A: (Tailored workup - not "shotgun"):
  • All patients: FBC, ESR, CRP, RFTs, LFTs, serum ACE + lysozyme, RPR/VDRL + FTA-ABS (syphilis screen), IGRA or Mantoux + CXR (TB), toxoplasma IgG/IgM
  • Based on clinical clues: HLA-B27 (AS/ReA), HLA-A29 (birdshot), HLA-B51 (Behcet), ANA/anti-dsDNA (SLE), ANCA (vasculitis), Toxocara ELISA, Lyme serology, Bartonella serology, HIV
  • Imaging: FFA (fluorescein angiography), ICG angiography, OCT (macula + retinal nerve fiber layer), B-scan ultrasound
  • Advanced: Aqueous/vitreous PCR (HSV/VZV/CMV/toxoplasma), vitreous cytology (lymphoma), MRI brain (lymphoma/MS), lumbar puncture (VKH, syphilis, lymphoma)
Q10. What is the treatment principle for non-infectious posterior uveitis? A: The mainstay is corticosteroids (systemic, periocular, or intravitreal) to suppress inflammation. The step-ladder approach:
  1. Topical steroids (anterior spillover)
  2. Periocular steroids (sub-Tenon triamcinolone) for unilateral posterior uveitis
  3. Systemic prednisone 1 mg/kg/day for bilateral or vision-threatening cases
  4. Steroid-sparing immunosuppressants (Methotrexate, Azathioprine, Mycophenolate, Cyclosporin)
  5. Biologics for refractory disease (Adalimumab, Infliximab, Rituximab)
Q11. Why must you NEVER start steroids without ruling out infection in posterior uveitis? A: Infectious causes (especially toxoplasmosis, TB, syphilis, fungal, viral) can be dramatically worsened by corticosteroids. Steroids suppress the immune response that is partially containing the infection, leading to uncontrolled microbial proliferation, worsening retinal destruction, and potentially blinding or life-threatening consequences. This is particularly critical for TB and CMV.
Q12. What is the ocular emergency associated with posterior uveitis? A: Several - (a) ARN with risk of bilateral blindness/retinal detachment; (b) CMV retinitis in HIV (rapid expansion threatening macula); (c) Necrotizing fungal endophthalmitis; (d) PORN (rapidly progressive outer retinal necrosis); (e) Exudative retinal detachment in VKH (can cause acute vision loss). All require urgent ophthalmological intervention.
Q13. What is the difference between choroiditis and retinitis on examination? A: Retinitis produces white lesions (retinal layers appear white because of edema/necrosis of neural tissue); active lesions have indistinct hazy borders. Choroiditis produces yellow/cream lesions (due to choroidal involvement with overlying retinal pigment epithelium changes); active choroidal lesions are rounder, more distinct. Vitritis is more common with retinitis than isolated choroiditis.
Q14. Name masquerade syndromes of posterior uveitis. A: Conditions that mimic posterior uveitis:
  • Primary Intraocular Lymphoma (PIOL)
  • Retinoblastoma (in children)
  • Malignant melanoma of choroid
  • Leukemic retinopathy
  • Retinitis pigmentosa
  • Rhegmatogenous retinal detachment (Schwartz-Matsuo syndrome)
  • Amyloidosis
  • Asteroid hyalosis

MANAGEMENT PROTOCOL

Step 1: Confirm Diagnosis of Posterior Uveitis

  • Slit-lamp + dilated fundal examination (DFE) with indirect ophthalmoscopy
  • Confirm anatomical location (retinitis / choroiditis / vasculitis / neuroretinitis)
  • Document: lesion character (white vs. yellow), borders, distribution, vitritis grade, disc/macular involvement

Step 2: Baseline Assessment

  • Visual acuity (distance + near), IOP, anterior segment (rule out panuveitis/anterior component)
  • Fundal photography, OCT (macula - detect CME, subretinal fluid, RPE changes)
  • Fluorescein angiography (FFA) - defines vasculitis, leakage, ischaemia
  • ICG angiography - for choroidal lesions (better than FFA for choroid)
  • B-scan ultrasound if media opacity obscures view

Step 3: Targeted Investigations (Tailored, Not Shotgun)

Always:
  • Serum ACE + lysozyme (sarcoidosis)
  • RPR/VDRL + FTA-ABS (syphilis)
  • IGRA/QuantiFERON + CXR (TB)
  • Toxoplasma IgG/IgM
  • HIV test (CMV risk stratification)
As indicated by clinical picture:
  • HLA-B51 (Behcet), HLA-A29 (Birdshot), ANA/ANCA (SLE/vasculitis)
  • Lyme antibody, Bartonella serology, Toxocara ELISA
  • HSV/VZV/CMV aqueous PCR (herpes family suspected)
  • Vitreous cytology + IL-10:IL-6 ratio (lymphoma suspected)
  • MRI brain (lymphoma, MS, VKH)
  • Lumbar puncture (VKH, syphilis with neurological features)

Step 4: Rule Out Infection Before Treatment

CRITICAL RULE: NEVER start immunosuppressive therapy until infectious causes - particularly toxoplasmosis, syphilis, TB, CMV, and fungal infection - are excluded or being treated concurrently.

Step 5: Specific Treatment (Cause-Directed)

CauseSpecific Treatment
ToxoplasmosisPyrimethamine + Sulfadiazine + Folinic acid (or TMP-SMX) ± short-course systemic steroids
ARN (HSV/VZV)IV Acyclovir → oral Valacyclovir; prophylactic laser
CMV RetinitisValganciclovir PO or IV Ganciclovir; intravitreal implant
PORNIV Ganciclovir + Foscarnet (combination)
TB ChoroiditisHRZE regimen (6-9 months) + adjunctive systemic steroids
Ocular SyphilisIV Penicillin G (neurosyphilis dosing, 14 days)
Candida endophthalmitisIntravitreal Voriconazole + systemic antifungal
ToxocariasisSteroids + Albendazole; vitrectomy for traction

Step 6: Anti-Inflammatory Therapy (Non-Infectious / Inflammatory)

Step-Ladder Approach:
Step A - Local Treatment (unilateral, mild-moderate)
  • Topical steroids (prednisolone 1% / dexamethasone 0.1%) for anterior spillover
  • Sub-Tenon triamcinolone acetonide (40 mg in 1 mL) - first-line for unilateral posterior uveitis; targets posterior segment directly
  • Intravitreal triamcinolone (4 mg) - for CME/severe inflammation in selected cases
  • Intravitreal dexamethasone implant (Ozurdex 0.7 mg) - sustained release for non-infectious uveitis
  • Cycloplegics: Atropine 1% or Cyclopentolate to prevent posterior synechiae
Step B - Systemic Corticosteroids (bilateral, severe, or vision-threatening)
  • Oral Prednisolone: 1-1.5 mg/kg/day (maximum 60-80 mg/day), then taper slowly over weeks-months
  • IV Methylprednisolone 500-1000 mg/day for 3 days (pulse therapy) for acute vision-threatening disease (e.g., VKH, acute ARN, optic disc involvement)
  • Gastroprotection (PPI), calcium + Vitamin D supplementation, bone protection (bisphosphonates if >3 months)
Step C - Steroid-Sparing Immunosuppressants (chronic/bilateral/steroid-dependent) Initiated when steroid dose cannot be tapered below 10 mg/day, or after 3+ months of high-dose steroid:
  • Antimetabolites (first-line): Methotrexate (7.5-25 mg/week PO) or Mycophenolate mofetil (1-3 g/day) - preferred for most chronic non-infectious posterior uveitis
  • T-cell inhibitors: Cyclosporin A (2-5 mg/kg/day) - preferred for Behcet, VKH, Birdshot
  • Alkylating agents: Cyclophosphamide or Chlorambucil for severe necrotizing disease (rare, high toxicity)
Step D - Biologics (Refractory / Steroid-Dependent)
  • Adalimumab (Humira) - Anti-TNF-α; FDA-approved for non-infectious intermediate/posterior/panuveitis (VISUAL I and II trials). First-line biologic.
  • Infliximab - Anti-TNF-α; particularly effective for Behcet disease ocular involvement
  • Rituximab - Anti-CD20; for PIOL (intravitreal or systemic)
  • Interferon-alpha 2a - For Behcet disease-associated uveitis; effective but side-effect profile limits use

Step 7: Manage Complications

ComplicationManagement
Cystoid macular oedema (CME)Topical NSAIDs, intravitreal steroid/anti-VEGF, treat underlying uveitis
Secondary glaucomaIOP-lowering drops (avoid prostaglandins in active uveitis), trabeculectomy if refractory
Complicated cataractDefer until uveitis quiescent for ≥3 months; phacoemulsification + IOL
Retinal detachmentVitrectomy + silicone oil (especially ARN, PORN)
Choroidal neovascular membrane (CNVM)Intravitreal anti-VEGF (bevacizumab/ranibizumab)
Vitreous hemorrhageObservation; vitrectomy if non-resolving
Disc / macular scarringLow vision rehabilitation

Step 8: Monitoring and Follow-Up

  • Active disease: Every 1-4 weeks (depending on severity)
  • Stable/maintenance: Every 3-6 months
  • Monitor: VA, IOP, vitreous haze (standardized SUN grading), fundal photography, OCT (CME detection), ERG (Birdshot - for subclinical disease progression)
  • Assess for steroid/immunosuppressant side effects at every visit
  • Target: Complete suppression of all anterior chamber and vitreous inflammation (zero cells) - even low-grade ongoing activity accelerates complications
Key References:
  • Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Ed. (Bowling)
  • The Wills Eye Manual, 8th Ed.
  • Firestein & Kelley's Textbook of Rheumatology
  • SUN Working Group Classification Criteria for Uveitis
This is a shared conversation. Sign in to Orris to start your own chat.