Sarcoid Uveitis

Ocular sarcoidosis occurs in about 25 to 60 percent of patients with systemic sarcoidosis (granulomatous multisystem disease), and uveitis may be the first manifestation. It is a major cause of granulomatous uveitis and panuveitis.

• Peak age 20 to 40 years and a second peak after 50 in women; higher prevalence and severity in people of African ancestry; bilateral in about 70 to 80 percent.
• Pathology: non-caseating granulomas with CD4 T-lymphocyte accumulation.
• Subtypes: acute anterior uveitis (Lofgren syndrome, with erythema nodosum, bilateral hilar lymphadenopathy, and arthralgia; usually self-limited), chronic granulomatous anterior uveitis, intermediate uveitis, posterior segment disease, and panuveitis. Heerfordt syndrome: uveitis, parotid enlargement, facial palsy, fever.

• Anterior: mutton fat keratic precipitates, iris nodules (Koeppe at the pupil margin, Busacca on the iris surface), posterior synechiae, conjunctival nodules, glaucoma, band keratopathy, lacrimal gland enlargement and dry eye.
• Intermediate: snowballs and snowbanks, string-of-pearls vitreous opacities.
• Posterior: periphlebitis with perivenous exudates ("candle wax drippings"), choroidal granulomas, macroaneurysms, retinal neovascularisation, disc granuloma, disc oedema, optic neuropathy, cystoid macular oedema.
• Systemic: cough, dyspnoea, hilar lymphadenopathy, skin lesions (erythema nodosum, lupus pernio), joint pain, fatigue, hypercalcaemia, neurological, cardiac (arrhythmia) and renal involvement.

• Slit lamp and dilated fundus examination, optical coherence tomography, widefield fluorescein angiography.
• Chest X-ray and high resolution CT chest (bilateral hilar lymphadenopathy, perilymphatic nodules); serum ACE, lysozyme, soluble interleukin-2 receptor, calcium, 24-hour urinary calcium, liver enzymes, creatinine; interferon gamma release assay and syphilis serology to exclude mimics.
• Whole body gallium or fluorodeoxyglucose positron emission tomography-CT when biopsy target is uncertain.
• Tissue biopsy (conjunctival nodule, lymph node by endobronchial ultrasound, lacrimal gland, skin) showing non-caseating granulomas confirms the diagnosis. The diagnosis is supported by bilateral hilar lymphadenopathy together with compatible ocular signs.
• Electrocardiogram, pulmonary function tests, and referral to respiratory physician.

• Anterior uveitis: prednisolone acetate 1 percent four to eight times daily tapered over several weeks, cycloplegic, and periocular steroid for resistant disease.
• Posterior, intermediate or panuveitis or macular oedema: sub-Tenon triamcinolone 40 mg, intravitreal dexamethasone implant 0.7 mg, or oral prednisolone 0.5 to 1 mg/kg daily for 4 to 6 weeks, tapering to 5 to 10 mg daily.
• Steroid sparing agents early for chronic disease: methotrexate 15 to 25 mg weekly, mycophenolate 1 g twice daily, azathioprine 2 mg/kg daily, adalimumab 80 mg then 40 mg every 2 weeks, or infliximab 5 mg/kg (preferred for refractory or neuro ocular disease).
• Treat complications: glaucoma (aqueous suppressants, drainage device), cataract surgery after 3 months of quiet inflammation, retinal neovascularisation (laser photocoagulation), band keratopathy, and dry eye.
• Systemic co-management with respiratory, cardiology and neurology teams; screen for hypercalcaemia, avoid vitamin D and calcium supplementation without monitoring, and provide bone protection with long term steroid.
• Follow up every 3 to 6 months, as relapse is common during tapering.