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Uveitis Caused by Connective Tissue Disease

by Emmett T. Cunningham, Jr., MD, PhD, MPH

A number of connective tissue diseases cause inflammation of the uveal tract.

Spondyloarthropathies

The seronegative spondyloarthropathies (see Overview of Seronegative Spondyloarthropathies) are a common cause of anterior uveitis. RA, in contrast, is not associated with isolated uveitis but can cause scleritis, which may cause secondary uveal tract inflammation. Ocular inflammation is most common with ankylosing spondylitis but also occurs with reactive arthritis, inflammatory bowel disease (ulcerative colitis and Crohn disease), and psoriatic arthritis. Uveitis is classically unilateral, but recurrences are common and active inflammation may alternate between eyes. Men are affected more commonly than women. Most patients, regardless of sex, are HLA-B27 positive.

Treatment requires a topical corticosteroid and a cycloplegic-mydriatic drug. Occasionally, periocular corticosteroids are required. Severe chronic cases may require noncorticosteroid immunosuppressive drugs (eg, methotrexate, mycophenolate mofetil).

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

JIA characteristically causes chronic bilateral iridocyclitis in children, particularly those with the pauciarticular variety (see Juvenile Idiopathic Arthritis (JIA)). Unlike most forms of anterior uveitis, however, JIA tends not to cause pain, photophobia, and conjunctival injection but only blurring and meiosis and is, therefore, often referred to as white iritis. JIA-associated uveitis is more common among girls.

Recurrent bouts of inflammation are best treated with a topical corticosteroid and a cycloplegic-mydriatic drug. Long-term control often requires use of a noncorticosteroid immunosuppressive drug (eg, methotrexate, mycophenolate mofetil).

Sarcoidosis

Sarcoidosis (see also Sarcoidosis) accounts for 10 to 20% of cases of uveitis, and about 25% of patients with sarcoidosis develop uveitis. Sarcoid uveitis is more common among blacks and the elderly.

Virtually any symptoms and signs of anterior, intermediate, posterior, or panuveitis can occur. Suggestive findings include conjunctival granulomas, large keratic precipitates on the corneal endothelium (so-called granulomatous or mutton fat precipitates), iris granulomas, and retinal vasculitis. Biopsy of suggestive lesions, which provides the most secure diagnosis, is usually done on the conjunctiva; it is rarely done on intraocular tissues because of the risk associated with the procedure.

Treatment usually involves topical, periocular, intraocular, or systemic corticosteroids, or a combination, along with a topical cycloplegic-mydriatic drug. Patients with moderate to severe inflammation may require a noncorticosteroid immunosuppressive drug (eg, methotrexate, mycophenolate mofetil, azathioprine).

Behçet disease

Behçet disease is rare in North America but is a fairly common cause of uveitis in the Middle East and Far East (see also Behçet Disease).

Typical findings include severe anterior uveitis with hypopyon, retinal vasculitis, and optic disk inflammation. The clinical course is usually severe with multiple recurrences.

Diagnosis requires the presence of associated systemic manifestations, such as oral aphthous or genital ulcers; dermatitis, including erythema nodosum; thrombophlebitis; or epididymitis. Oral aphthae may be biopsied to show an occlusive vasculitis. There are no laboratory tests for Behçet disease.

Treatment with local and systemic corticosteroids and a cycloplegic-mydriatic drug may alleviate acute exacerbations, but most patients eventually require systemic corticosteroids and a noncorticosteroid immunosuppressive drug (eg, cyclosporine, chlorambucil) to control the inflammation and avoid the serious complications of long-term corticosteroid treatment. Biologic agents such as interferons and tumor necrosis factor inhibitors have been effective in selected patients unresponsive to other therapies.

Vogt-Koyanagi-Harada (VKH) disease

VKH disease is an uncommon systemic disorder characterized by uveitis accompanied by cutaneous and neurologic abnormalities. VKH disease is particularly common among people of Asian, Asian Indian, and American Indian descent. Women in their 20s and 30s are affected more often than men. The etiology is unknown, although an autoimmune reaction directed against melanin-containing cells in the uveal tract, skin, inner ear, and meninges is strongly suspected.

Neurologic symptoms tend to occur early and include tinnitus, dysacusis (auditory agnosia), vertigo, headache, and meningismus. Cutaneous findings frequently occur later and include patchy vitiligo (especially common on the eyelids, low back, and buttocks), poliosis (a localized patch of white hair), and alopecia, often involving the head and neck. Common findings include serous retinal detachment, optic disk edema, and choroiditis. Long-term complications include cataracts, glaucoma, subretinal fibrosis, and choroidal neovascularization.

Early treatment includes local and systemic corticosteroids and a cycloplegic-mydriatic drug. Many patients also require noncorticosteroid immunosuppressive drugs (eg, methotrexate, azathioprine, mycophenolate mofetil).

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