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Uveitis Caused by Connective Tissue Disease

By

Kara C. LaMattina

, MD, Boston University School of Medicine

Last full review/revision Sep 2022| Content last modified Sep 2022
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Spondyloarthropathies

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)

JIA Juvenile Idiopathic Arthritis (JIA) Juvenile idiopathic arthritis is a group of rheumatic diseases that begins by age 16. Arthritis, fever, rash, adenopathy, splenomegaly, and iridocyclitis are typical of some forms. Diagnosis... read more Juvenile Idiopathic Arthritis (JIA) , previously known as juvenile RA, characteristically causes chronic bilateral iridocyclitis in children, particularly those with the pauciarticular variety. Unlike most forms of anterior uveitis, however, JIA-associated uveitis tends not to cause pain, photophobia, and conjunctival injection but only blurring and miosis and is, therefore, often referred to as white iritis. It can be asymptomatic. JIA-associated uveitis is more common among girls. Because symptoms can be overlooked or absent, patients with JIA should be regularly screened.

Rheumatoid arthritis, in contrast, is not associated with isolated uveitis but can cause scleritis, which may cause secondary uveal tract inflammation.

Recurrent bouts of inflammation are best treated with a topical corticosteroid and a cycloplegic-mydriatic drug. Given the chronic nature of disease and risk of treatment-related cataract and glaucoma development, long-term control often requires use of a noncorticosteroid immunosuppressive drug (eg, methotrexate, mycophenolate mofetil).

Sarcoidosis

Sarcoidosis Sarcoidosis Sarcoidosis is an inflammatory disorder resulting in noncaseating granulomas in one or more organs and tissues; etiology is unknown. The lungs and lymphatic system are most often affected, but... read more 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 patients of African descent and older patients.

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 or chronic disease may require a noncorticosteroid immunosuppressive drug (eg, methotrexate, mycophenolate mofetil).

Behçet disease

Typical findings include severe anterior uveitis with hypopyon, retinitis, 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, but it is associated with HLA-B51.

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 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. Alkylating agents (eg, cyclosporine and chlorambucil) have induced remission in patients.

Vogt-Koyanagi-Harada (VKH) disease

Vogt-Koyanagi-Harada 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, American Indian, and Hispanic 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, which may involve the eyelashes), and alopecia, often involving the head and neck. Common findings include serous retinal detachment Retinal Detachment Retinal detachment is separation of the neurosensory retina from the underlying retinal pigment epithelium. The most common cause is a retinal break (a tear or, less commonly, a hole—rhegmatogenous... read more Retinal Detachment , optic disk edema, and choroiditis. Long-term complications include cataracts Cataract A cataract is a congenital or degenerative opacity of the lens. The main symptom is gradual, painless vision blurring. Diagnosis is by ophthalmoscopy and slit-lamp examination. Treatment is... read more Cataract , glaucoma Overview of Glaucoma Glaucomas are a group of eye disorders characterized by progressive optic nerve damage in which an important part is a relative increase in intraocular pressure (IOP) that can lead to irreversible... read more , 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, mycophenolate mofetil).

Tubulointerstitial nephritis and uveitis syndrome (TINU)

TINU typically presents with nongranulomatous acute bilateral anterior uveitis, though it is frequently accompanied by posterior complications, including edema of the optic nerve and macula. It most commonly affects adolescent females but can present at any age. Symptoms include eye pain and redness, decreased visual acuity, and photophobia. Patients may have a history of viral prodrome symptoms and also flank pain, polyuria, and nocturia. Evaluation for nephritis Tubulointerstitial Nephritis Tubulointerstitial nephritis is primary injury to renal tubules and interstitium resulting in decreased renal function. The acute form is most often due to allergic drug reactions or to infections... read more Tubulointerstitial Nephritis may include urine beta-2 microglobulin levels and sometimes renal biopsy Renal biopsy Biopsy of the urinary tract requires a trained specialist (nephrologist, urologist, or interventional radiologist). Indications for diagnostic biopsy include unexplained nephritic or nephrotic... read more . While the acute kidney injury Acute Kidney Injury (AKI) Acute kidney injury is a rapid decrease in renal function over days to weeks, causing an accumulation of nitrogenous products in the blood (azotemia) with or without reduction in amount of urine... read more is frequently self-limited in TINU, the ocular disease is often chronic (1 TINU reference A number of connective tissue diseases cause inflammation of the uveal tract. (See also Overview of Uveitis.) The seronegative spondyloarthropathies are a common cause of anterior uveitis. Among... read more TINU reference ).

Treatment with oral steroids in the acute phase and use of noncorticosteroid immunosuppressive drugs (eg, mycophenolate mofetil) for chronic disease is often necessary.

TINU reference

  • 1. Koreishi AF, Zhou M, Goldstein DA: Tubulointerstitial nephritis and uveitis syndrome: Characterization of clinical features. Ocul Immunol Inflamm 17;29(7-8):1312-1317, 2021. doi: 10.1080/09273948.2020.1736311

Drugs Mentioned In This Article

Drug Name Select Trade
OTREXUP
CELLCEPT
NEORAL, SANDIMMUNE
LEUKERAN
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