title: Ocular Inflammatory Disorders in Autoimmune Lymphoproliferative Syndrome (ALPS).
abstract: OBJECTIVE
  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.
authors.affiliation: a National Eye Institute, National Institutes of Health , Bethesda , Maryland , USA.|b Department of Ophthalmology , Cerrahpasa Medical School, University of Istanbul , Istanbul , Turkey.
authors.forename: Didar
authors.initials: D
authors.lastname: Ucar
authors.affiliation: a National Eye Institute, National Institutes of Health , Bethesda , Maryland , USA.|c University of California, San Diego, School of Medicine , La Jolla , California , USA.
authors.forename: Jane S
authors.initials: JS
authors.lastname: Kim
authors.affiliation: a National Eye Institute, National Institutes of Health , Bethesda , Maryland , USA.
authors.forename: Rachel J
authors.initials: RJ
authors.lastname: Bishop
authors.affiliation: a National Eye Institute, National Institutes of Health , Bethesda , Maryland , USA.
authors.forename: Robert B
authors.initials: RB
authors.lastname: Nussenblatt
authors.affiliation: d National Institute of Allergy and Infectious Diseases, National Institute of Health , Bethesda , Maryland , USA.
authors.forename: V Koneti
authors.initials: VK
authors.lastname: Rao
authors.affiliation: a National Eye Institute, National Institutes of Health , Bethesda , Maryland , USA.
authors.forename: H Nida
authors.initials: HN
authors.lastname: Sen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D056735:Autoimmune Lymphoproliferative Syndrome / Q000150:complications* / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009902:Optic Neuritis / Q000175:diagnosis / Q000209:etiology*; D012189:Retrospective Studies; D014605:Uveitis / Q000175:diagnosis / Q000209:etiology*; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
keywords: ALPS; autoimmune lymphoproliferative syndrome; eye findings; ocular inflammation; uveitis