Is there a predisposition to uveitis in Turner syndrome?

Abstract

Introduction: Autoimmunity is prevalent in Turner syndrome (TS) though uveitis is rarely reported. A definite link between TS and uveitis is not yet established.

Methods: We report two cases of uveitis with a history of TS and review the literature regarding TS, uveitis and autoimmunity.

Results: TS-associated uveitis is acute (100%), non-hypertensive (100%) anterior uveitis (87.5%) that usually responds to topical therapy without unexpected long-term visual sequelae. Systemic treatment is uncommonly required as relapses are infrequent.

Conclusion: Reported cases of uveitis in TS were acute/symptomatic, normotensive and both unilateral and bilateral cases have been described. Systemic causes including infectious (e.g. syphilis, tuberculosis), noninfectious (e.g. sarcoidosis, HLA-B27) and specific syndromes (e.g. tubulointerstitial nephritis with uveitis, juvenile idiopathic arthritis) should be sought. Systemic immunosuppression was not needed in most cases as a good response to topical therapy was typical. There are baseline risks in TS (e.g. further growth limitation in children, baseline increased risk of solid tumors, diabetes mellitus), which should be considered before commencing systemic corticosteroids or immunosuppressants.