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Bilateral uveitis in a patient with autoimmune lymphoproliferative syndrome
Wee-Kiak Lim1, Roxana Ursea, Koneti Rao
1Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892-1857, USA.
Purpose:
We report a case of autoimmune lymphoproliferative syndrome (ALPS) presenting with bilateral uveitis.
Design:
Observational case report.
Methods:
Review of case record, serum and aqueous IL-10 and IL-6 cytokine results, and immunosuppressive treatment of a patient with a mutation in the gene encoding Fas.
Results:
Control of the intermediate uveitis required sustained doses of topical and periocular corticosteroids as well as systemic cyclosporine. The serum IL-10 level was elevated, as commonly seen in ALPS, but the aqueous IL-10 was not.
Conclusions:
Despite a Th2 immune predominance in ALPS, uveitis, a Th1-mediated disease, may still manifest in these patients. The pathogenesis of uveitis in ALPS may differ from that of the systemic disease overall. Long-term follow-up is required for patients with uveitis associated with ALPS.
