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Ocular Inflammatory Disorders in Autoimmune Lymphoproliferative Syndrome (ALPS) |
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| Authors: | Didar Ucar Jane S Kim Rachel J Bishop Robert B Nussenblatt V Koneti Rao |
| Institution: | 1. National Eye Institute, National Institutes of Health, Bethesda, Maryland, USA;2. Department of Ophthalmology, Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey;3. University of California, San Diego, School of Medicine, La Jolla, California, USA;4. National Institute of Allergy and Infectious Diseases, National Institute of Health, Bethesda, Maryland, USA |
| Abstract: | Purpose: To describe inflammatory ocular findings in patients with autoimmune lymphoproliferative syndrome (ALPS). Methods: A retrospective review of medical records for ALPS patients seen at the National Eye Institute between 2003 and 2013. Results: A total of 29 ALPS patients previously referred for ocular or visual symptoms or with a history of prolonged corticosteroid use, were identified. Mean age was 20 years (range: 4–66 years). The majority were male (n = 21, 72.4%) and Caucasian (n = 24, 82.8%). Ten (34.5%) had abnormal ocular findings, the most common of which was an ocular inflammatory disorder (n = 4, 13.8%). Uveitis was seen in two patients with ALPS-FAS and one with ALPS-U, all of whom required long-term systemic immunosuppression. One patient with ALPS-FAS had a history of optic neuritis. Conclusions: ALPS can have intraocular inflammatory manifestations that require routine follow-up to ensure appropriate and timely treatment of intraocular disease. Long-term immunosuppression may be needed for patients with ALPS-associated uveitis. |
| Keywords: | ALPS autoimmune lymphoproliferative syndrome eye findings ocular inflammation uveitis |