Bilateral uveitis in a patient with autoimmune lymphoproliferative syndrome

Wee Kiak Lim, Roxana Ursea, Koneti Rao, Ronald R. Buggage, Eric B. Suhler, Faith Dugan, Chi Chao Chan, Stephen E. Straus, Robert B. Nussenblatt

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)562-563
Number of pages2
JournalAmerican journal of ophthalmology
Volume139
Issue number3
DOIs
StatePublished - Mar 2005

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ASJC Scopus subject areas

  • Ophthalmology

Cite this

Lim, W. K., Ursea, R., Rao, K., Buggage, R. R., Suhler, E. B., Dugan, F., Chan, C. C., Straus, S. E., & Nussenblatt, R. B. (2005). Bilateral uveitis in a patient with autoimmune lymphoproliferative syndrome. American journal of ophthalmology, 139(3), 562-563. https://doi.org/10.1016/j.ajo.2004.09.017