Uveitis due to connective tissue diseases

A group of connective tissue diseases cause inflammation of the uveal tract, resulting in uveitis.

Spondyloarthropathy

Seronegative spondyloarthropathies are a common cause of anterior uveitis. Inflammation of the eye most often occurs in ankylosing spondylitis, as well as in reactive arthritis and psoriatic arthritis; gastrointestinal inflammation, which includes ulcerative colitis and Crohn's disease. Uveitis is usually unilateral, but relapses are common, which may affect one eye or the other. Men are more often affected than women. Most patients, regardless of gender, are HLA-B27-positive. Treatment requires topical glucocorticoids and mydriatics. Sometimes it is necessary to administer glucocorticoids parabulbarly.

Juvenile idiopathic arthritis (JIA, also known as juvenile RA)

Juvenile rheumatoid arthritis causes chronic bilateral iridocyclitis in children. Unlike most forms of anterior uveitis, however, JIA does not tend to cause pain, photophobia, and conjunctival injection, but only blurred vision and miosis and is therefore often referred to as "white" iritis. JIA uveitis occurs more frequently in girls than in boys. Recurrent attacks of inflammation are best treated with a topical glucocorticoid and a cycloplegic-mydriatic medication. Long-term control often requires the use of a nonsteroidal immunosuppressant such as methotrexate or mucophenolate modefil.

Sarcoidosis

Sarcoidosis accounts for 10-25% of uveitis cases, and approximately 25% of patients with sarcoidosis develop uveitis. Sarcoidosis uveitis is most common in blacks and the elderly. Virtually any of the symptoms and signs of anterior, middle, and posterior uveitis may develop. The hallmarks of sarcoidosis uveitis are conjunctival granulomas, large corneal precipitates on the corneal endothelium (called granulomatous precipitates), iris granulomas, and retinal vasculitis. Biopsy of suspected lesions provides the most reliable diagnosis. Treatment usually involves topical, periocular, and systemic glucocorticoids along with a mydriatic. Patients with moderate to severe inflammation may require a nonsteroidal immunosuppressant such as methotrexate, mycophenolate modefil, or azathioprine.

Behcet's syndrome

This disease is rare in North America but is a common cause of uveitis in the Middle and Far East. The typical clinical picture consists of severe anterior uveitis with hypopyon (a collection of white blood cells in the anterior chamber), retinal vasculitis, and optic disc inflammation. The clinical course is usually severe with multiple relapses. Diagnosis requires the presence of associated systemic manifestations such as aphthous stomatitis and genital ulcers; dermatitis including erythema nodosum; thrombophlebitis, or epididymitis. Oral aphthous lesions and genital ulcers may be biopsied to demonstrate occlusive vasculitis. There are no laboratory tests for Behcet's syndrome. Treatment with local or systemic glucocorticoids and mydriatics may reduce manifestations, but most patients require systemic glucocorticoids and nonsteroidal immunosuppressants such as cyclosporine or chlorambucil to control inflammation and avoid serious complications of long-term glucocorticoid use.

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Vogt-Koyanagi-Harada syndrome (VKH)

Vogt-Koyanagi-Harada syndrome is a rare systemic disorder characterized by uveitis in association with cutaneous and neurologic abnormalities. Vogt-Koyanagi-Harada syndrome is particularly common in people of Asian, Asian-Indian, and American-Indian descent. Women in their 20s and 30s are more commonly affected than men. The etiology is unknown, although an autoimmune reaction directed against melanin-containing cells in the uveal tract, skin, inner ear, and meningeal membranes is suspected.

Neurological symptoms tend to appear early and include tinnitus, hearing loss (auditory agnosia), dizziness, headache, and meningismus. Skin manifestations tend to appear later and include vitiligo (especially common on the eyelids, lower back, and buttocks), poliosis (graying of the eyelashes), and alopecia (hair loss). Additional ocular complications include cataracts, glaucoma, papilledema, and choroiditis, often with exudative retinal detachment.

Early therapy consists of topical and systemic glucocorticoids and mydriatics. Many patients also require a non-glucocorticoid immunosuppressant such as methotrexate, azathioprine, or mucophenolate modefil.