Ocular Inflammatory Disorders in Autoimmune Lymphoproliferative Syndrome (ALPS).

Ucar D. , Kim J. S. , Bishop R. J. , Nussenblatt R. B. , Rao V. K. , Sen H. N.

Ocular immunology and inflammation, vol.25, no.5, pp.703-709, 2017 (Journal Indexed in SCI Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 25 Issue: 5
  • Publication Date: 2017
  • Doi Number: 10.1080/09273948.2016.1175637
  • Title of Journal : Ocular immunology and inflammation
  • Page Numbers: pp.703-709


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.