Ocular involvement sarcoidosis

by Nathan Wei, MD, FACP, FACR

Nathan Wei is a nationally known board-certified rheumatologist and author of the Second Opinion Arthritis Treatment Kit. It's available exclusively at this website... not available in stores.

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Sarcoidosis is a systemic granulomatous disease. The cause is unknown but it does involve autoimmune dysfunction. It may present as a debilitating, febrile illness with cough and shortness of breath, fatigue, bilateral (occurring on both sides) hilar lymphadenopathy (enlarged lymph nodes in the center of the chest- this is visible on a plain chest x-ray), erythema nodosum (painful bumps on the shins), alveolitis (inflammation of air sacks in the lungs), acute polymyositis (inflammatory muscle disease), arthritis, lacrimal or salivary gland infiltration (inflammation of the glands that make tears and saliva) or sarcoid nodules of the skin.

It occurs most frequently in young adults (20 to 40 years), and favors women. It is more common in people of Scandinavian descent and also in African Americans.

Patients diagnosed with systemic sarcoidosis have a nearly 20 percent incidence of eye (ocular) involvement. The most common ocular sign is unilateral (occurring on one side only), anterior, granulomatous uveitis.

The common clinical ocular findings associated with sarcoid uveitis include decreased or hazy vision, pain, photophobia (painful eyes with exposure to light), lacrimation (teary eyes), and conjunctival injection (red eye). Membranes can form across the eye. Also, cataracts, glaucoma, optic nerve inflammation, bulging of the eye, hemorrhages of the retina, and paralysis of the muscles that move the eye may all occur.

The underlying pathophysiology favors the interaction of lymphocytes with macrophages. Some postulate that CD4+ T-helper 1 cells, in concert with macrophages, produce a cascade of cytokines and chemotactic factors which result in tissue changes and granulomatous lesions that affect many tissues and allow for the multi-system nature of this disease. The clinical features of sarcoidosis mimic those of many rheumatologic diseases, with increasing reports of coexistent autoimmune disease.

Extensive laboratory testing is essential, especially for atypical uveitis or optic neuropathy. Obtain testing for anemia, leukemia, syphilis, HIV, lupus, Lyme disease, tuberculosis, arthritis, ankylosing spondylitis and hypertension. The initial tests should include complete blood count (CBC with differential), purified protein derivative with anergy panel (PPD with anergy panel), anti-nuclear antibody (ANA), angiotensin converting enzyme (ACE), Lyme titer, rheumatoid factor (RF), sickle cell prep, chest x-ray (CXR) and sacroiliac joint films. Tests that indicate sarcoidosis most specifically are the chest x-ray, ACE and gallium scan.

Management of the eye disease depends on the underlying problem. Obviously, close ophthalmologic surveillance is indicated. Topical steroids along with systemic anti inflammatory therapy consisting of either non-steroidal anti inflammatory drugs and oral steroids are used. In stubborn cases, methotrexate and cyclosporine have been used. With retinal involvement, a retina specialist needs to be consulted.

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