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Clinical Presentation
- Chronic, idiopathic, granulomatous multisystem disease, primarily affecting the lungs and lymph nodes
- Usually insidious onset with lung involvement (cough, dyspnea) and extrapulmonary manifestations (skin lesions such as erythema nodosum, lupus pernio; neurological diseases such as unilateral facial palsy, meningitis; arthritis, bone cysts, kidney disease)
- Special forms with acute onset:
- Löfgren Syndrome: Erythema nodosum and bilateral hilar lymphadenopathy, possibly accompanied by fever, anorexia, and arthralgia
- Heerfordt Syndrome: Uveitis, parotitis, fever, and cranial nerve palsies, usually involving the facial nerve
- Eye findings: Conjunctival granulomas, acute/chronic anterior uveitis, intermediate uveitis, periphlebitis (retinitis only affects venous segments!), choroidal infiltrates, multifocal choroiditis, retinal granulomas, peripheral retinal neovascularisation, optic nerve involvement (due to focal granulomas/papilledema/persistent optic disc swelling)
Work-up
- Chest X-ray or CT
- Biopsy of the lung, lymph nodes, conjunctiva, or lacrimal gland
- Laboratory: ACE, lysozyme, PPD (purified protein derivative)
- Additional tests: bronchoalveolar lavage, pulmonary function tests
Treatment
- Local/periocular/systemic steroids (e.g., tapering 60mg over 2 months)
- In case of recurrence, consider evaluating immunosuppressants (baseline therapy) such as methotrexate, azathioprine, cyclosporine
Sources
- EyeWiki Sarcoid Uveitis
- The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
- Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)