Bilateral uveitis in a patient with autoimmune lymphoproliferative syndrome.
OBJECTIVE
We report a case of autoimmune lymphoproliferative syndrome (ALPS) presenting with bilateral uveitis.


METHODS
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.
Lim
Wee-Kiak
WK
Laboratory of Immunology, National Eye Institute, National Institutes of Health, Bethesda, Maryland 20892-1857, USA.
Ursea
Roxana
R
Rao
Koneti
K
Buggage
Ronald R
RR
Suhler
Eric B
EB
Dugan
Faith
F
Chan
Chi-Chao
CC
Straus
Stephen E
SE
Nussenblatt
Robert B
RB
D001082:Aqueous Humor / Q000378:metabolism; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D002648:Child; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016753:Interleukin-10 / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008232:Lymphoproliferative Disorders / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D009154:Mutation; D018417:Th1 Cells / Q000276:immunology; D015867:Uveitis, Intermediate / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D019014:fas Receptor / Q000235:genetics
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D015850:Interleukin-6; D019014:fas Receptor; D016753:Interleukin-10