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Anterior Uveitis in Sarcoidosis - NEET PG Notes
The Big Picture
Sarcoidosis is a chronic granulomatous disorder of unknown cause characterized by non-caseating granulomas. It is one of the most common systemic associations of uveitis. Granulomatous anterior uveitis is its most common ocular manifestation. Ocular involvement occurs in 25-70% of sarcoid patients (varies by ethnicity).
- Black people are affected 10x more than White people
- More common in colder climates
- Uveitis can precede the diagnosis of sarcoidosis by decades
Anterior Uveitis Pattern in Sarcoidosis
| Feature | Detail |
|---|
| Type | Granulomatous anterior uveitis (most common) |
| Acuity | Both AAU (acute-onset sarcoidosis) and CAU (chronic - more common; tends to affect older patients with chronic pulmonary disease) |
| Laterality | Bilateral (usually) |
| KP type | "Mutton-fat" KPs - large, waxy, greasy deposits on corneal endothelium |
| KP distribution | Arlt's triangle - apex near central cornea, base at inferior limbus |
The 7 IWOS Signs (NEET High Yield!)
The International Workshop on Ocular Sarcoidosis (IWOS, 2009) identified 7 key signs for intraocular sarcoidosis diagnosis. Memorize these:
- Mutton-fat KPs and/or small granulomatous KPs and/or iris nodules (Koeppe and/or Busacca)
- Trabecular meshwork nodules and/or tent-shaped PAS (peripheral anterior synechiae)
- Vitreous opacities: snowballs and/or "strings of pearls"
- Multiple chorioretinal peripheral lesions (active/atrophic)
- Nodular/segmental periphlebitis ("candle-wax drippings" / en taches de bougie)
- Optic disc nodule(s)/granuloma(s) and/or solitary choroidal nodule
- Bilateral involvement
Iris Nodules - NEET Favorite
Three types of iris nodules in sarcoidosis (all are granulomas):
| Nodule | Location |
|---|
| Koeppe nodules | Pupillary border |
| Busacca (Bussaca) nodules | Iris stroma |
| Berlin nodules | Anterior chamber angle |
Note: Koeppe nodules are not specific to sarcoidosis - also seen in other granulomatous uveitis. But the triad with mutton-fat KPs is highly suggestive.
Slit-lamp Images of Granulomatous KPs
Mutton-fat keratic precipitates on the corneal endothelium - the hallmark of granulomatous uveitis such as sarcoidosis
Large, waxy mutton-fat KPs distributed in Arlt's triangle - inferior corneal endothelium
Structural Complications
- Posterior synechiae (iris stuck to lens)
- Cataract
- Cystoid macular edema (CME)
- Secondary glaucoma (trabecular meshwork involvement)
- Peripheral retinal neovascularization (from occlusive periphlebitis)
Systemic Features (Correlate with Ocular)
| System | Feature |
|---|
| Lung | Bilateral hilar lymphadenopathy (BHL), pulmonary fibrosis |
| Skin | Erythema nodosum (shins), lupus pernio (nose/cheeks/ears), granulomatous papules |
| Joints | Polyarthralgia |
| Nerve | Cranial nerve palsies (CN VII most common), meningitis |
| Heart | Arrhythmia, sudden death (5% clinically) |
| Kidney | Hypercalciuria |
Lofgren syndrome (acute, good prognosis): Erythema nodosum + BHL + polyarthralgia (triad; seen in women)
Investigations - NEET High Yield
| Test | Significance |
|---|
| Serum ACE (Angiotensin Converting Enzyme) | Elevated - most important marker |
| Serum lysozyme | Elevated |
| Chest X-ray | BHL (abnormal in 90%) |
| Tuberculin skin test | Negative (anergy) - strongly positive makes sarcoidosis unlikely |
| Serum calcium / hypercalciuria | Can be elevated |
| Bronchoalveolar lavage (BAL) | CD4/CD8 T-cell ratio elevated (>3.5 is suggestive) |
| Kveim test | Specific but rarely used now |
| Biopsy | Non-caseating granulomas (gold standard for definite diagnosis) |
| Conjunctival biopsy | Up to 75% positive if conjunctival nodules present |
Diagnostic Classification (IWOS)
| Category | Criteria |
|---|
| Definite ocular sarcoidosis | Biopsy-supported diagnosis + compatible uveitis |
| Presumed ocular sarcoidosis | No biopsy BUT chest X-ray shows BHL + compatible uveitis |
| Probable ocular sarcoidosis | No biopsy, no BHL on CXR, but other systemic/lab evidence |
| Possible ocular sarcoidosis | Negative systemic workup, non-caseating granuloma not found |
Treatment
| Severity | Treatment |
|---|
| Mild anterior uveitis | Topical corticosteroids + cycloplegics |
| Moderate/severe | Regional (periocular) or systemic corticosteroids |
| Steroid-resistant / chronic | Immunosuppressives (methotrexate, mycophenolate, azathioprine) |
| T-cell critical | Calcineurin inhibitors (tacrolimus, cyclosporine) |
| Refractory | Anti-TNF biologics (infliximab, adalimumab) |
Treatment activity does not always parallel systemic disease activity.
Quick-Fire NEET MCQ Points
- Most common ocular manifestation of sarcoidosis = Granulomatous anterior uveitis
- Most common manifestation overall = Dry eyes / lacrimal gland involvement
- KP type = Mutton-fat (granulomatous); NOT stellate
- Distribution = Arlt's triangle
- Iris nodule on pupillary border = Koeppe; on stroma = Busacca; in angle = Berlin
- Fundus finding = "Candle-wax drippings" (periphlebitis)
- Vitreous = "Strings of pearls" or snowballs
- Serum ACE = elevated; Tuberculin test = negative
- Chest X-ray = BHL (bilateral hilar lymphadenopathy) in 90%
- Biopsy shows = Non-caseating granulomas (vs TB = caseating)
- Posterior uveitis in sarcoid = frequently associated with CNS involvement
- Sarcoidosis can precede uveitis OR uveitis can precede sarcoidosis by decades
Differentials for Granulomatous Uveitis (Eliminate in MCQs)
| Condition | Differentiating clue |
|---|
| Tuberculosis | Caseating granuloma; positive tuberculin; iris pearls; sector PAS |
| Syphilis | VDRL/FTA-Abs positive; can mimic anything |
| VKH | Bilateral panuveitis; Dalen-Fuchs nodules; sunset glow fundus; poliosis |
| Sympathetic ophthalmia | History of penetrating trauma/surgery |
| Lepra (leprosy) | Iris pearls; beaded nerves |
| CMV uveitis | Immunocompromised; sectoral corneal edema |
Sources:
- Kanski's Clinical Ophthalmology: A Systematic Approach, 10th ed., pp. 456-475 (IWOS criteria, ocular features, investigations)
- Rheumatology, 2-Volume Set (Elsevier 2022), p. 82-93 (iris nodules, treatment principles)
- Goldman-Cecil Medicine, pp. 981-982 (epidemiology, posterior uveitis, CNS link)