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Sarcoidotic uveitis

Sarcoidosis is a multisystem granulomatous disease that commonly affects the lungs, skin, and lymph nodes. It causes non-caseating granulomas and can involve multiple organs including the eyes. Ocular involvement occurs in about 26% of chronic cases and most commonly manifests as chronic granulomatous anterior uveitis with mutton-fat keratic precipitates, iris nodules, and secondary glaucoma or cataracts. Posterior segment involvement such as choroidal or retinal lesions may also occur. The etiology is unknown but appears to involve an abnormal immune response triggered by environmental antigens in genetically susceptible individuals. Diagnosis requires biopsy demonstrating non-caseating granulomas and treatment

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Sarcoidotic Uveitis Bipin Bista Resident Ophthalmology National medical College & Teaching Hospital
• Multisystem granulomatous disease which affects every organ. • Lungs, thoracic LN & Skin – commonest • Adrenal glands are spared. • Initially, termed as skin disease – Hutchinson (1869) • Boeck termed it as sarcoid – biopsy. • Association with uveitis in 1900’s • Sarcoid uveitis associated with Facial Nerve palsy & uveoparotid fever : Heerfordt’s syndrome. • Epidemiology : Black > White, Common : Female. • Etiology : Unknown.
ETIOLOGY
Etiology • Macrophage :::: inflammatory response. • Release cytokines (TNF –α & IL-2) infiltration of T- helper lymphocytes release cytokines & recruit other inflammatory cells to site of inflammation. • In response, granulomas are formed & immunoglobulin produced in large quantities by stimulated B lymphocytes are sent into circulation. • Sensitization & resultant immune response to endogenous lung antigen or inhaled agents. • Environmental factors : molds & insecticides.
Clinical features • Asymptomatic. • Respiratory symptoms, fever, fatigue or weight loss. • Abnormal chest X-ray or laboratory tests. • Biopsy : Non-caseating,granulomatous,non-infectious inflammatory process. • 2/3rd of case – remission with in a decade. • 1/3rd of case - recurrent, progressive – significant organ damage. • Ocular involvement is about 26 % in chronic cases. • B/L but may be U/L as well as markedly asymmetric.
Clinical Manifestation
Ocular examination • Proper evaluation of lacrimal apparatus & conjunctiva. • Conjunctiva ( 7-17%) • Lacrimal gland (7-26%)
Anterior Uveitis • Most common • 2/3rd ( 50-60%) will have chronic granulomatous uveitis. • Most have Mutton fat KPs, large & dense, Iris nodules. • Inflammatory vs Malignancy. • Chronic form : 4th-6th decade & causes cataract (8-17%) & glaucoma (11-23%) with increase in duration • 15-45 % associated with acute iridocyclitis . • Mild vitritis ( occasional)
Posterior Segment • Less common . 6-33% • Clump of cells & proteinacous debris – snowballs • ‘Candle wax dripping’ (en taches de bongie) • Perivenous sheathing rarely periarterioral. • Deep yellow choroidal lesions consistent with D-F nodules & mottling of pigment epithelium (36%) • Serous RD – large granuloma • Chronic Cystoid Macular Oedema. • Neovascularization of Optic Disc (15%) • Retinal & subretinal neovascularization (R > SR) • Peripapillary or macular subretinal neovascular membrane.
Systemic Involvement • Liver (10% Liver enzyme) • Hypercalciuria • Child & infants. • Blau syndrome • Skin lesions are common. • Erythema nodusom & granulomatous sarcoid nodules • Colitis • Behςet’s Disease • Arthritis (Non- granulomatous synovitis) • Neurological involvement (25%), facial nerve. • Hilar adenopathy • Lymphadenopathy • Splenomegaly
PATHOLOGY • Sarcoid granuloma : whorls of epitheloid cells surrounding multinucleated giant cells, mononuclear cells, fibroblasts & lymphocytes. • Under polarised light : Asteroid Bodies, Schaumann’s body & crystalline inclusions. • Th1 driven inflammatory process , CD4+ T lymphocytes
Diagnosis • Requires biopsy • Asymptomatic • Abnormal chest x-ray. • 60% granuloma on transbronchial lung biopsy even with Normal radiography (Stage 0 disease) • 90% parenchymal involvement. • CT–Scan more useful as it’s more sensitive. • 27-55 %-Conjunctival biopsy • 22%-lacrimal biopsy
LABORATORY TESTS • ACE is pre-dominantly pulmonary macrophage & vascular endothelium ( elevated in 60-90%) • May be normal in quiescent disease. • ACE : TB, leprosy, silicosis, cirrhosis, asbestosis & histoplasmosis. • Present in aqueous humor & even in CSF. • Serum lysosymes • Hypercalciuria & hypercalcemia. • Lymphopenia & elevated liver enzyme. • KVEIN TEST : Spleen antigen : 6 weeks : 80% • Gallium scan • PFT • Limited diffusing capacity.
Treatment • Asymptomatic with hilar adenopathy (Doesn’t require) • 2 main indications : ―Symptomatic pulmonary disease ―Uveitis
• Acute anterior inflammation by corticosteroids but frequently peri-ocular steroid injection is required. • Chronic uveitis requires PULSE Therapy. • Immunosuppresants • Methotrexate : low dose effective & safe • Hydroxychloroquine • TNF-α Inhibitor : Infliximab • Pars plana lensectomy • ECCE • Laser photocoagulation : Subretinal neovascularization
GOALS • Control inflammatory condition • Prevent permanently visually impairing changes such as photoreceptor damage caused by Cystoid Macular Oedema.
Reference: - Nussenblatt and whitecup 4th edition - Myron yanoff 4th edition