Ocular Inflammatory Disorders in Autoimmune Lymphoproliferative Syndrome (ALPS)

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

DECLARATION OF INTEREST

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

FUNDING

This study was supported by the NEI and NIAID Intramural Research Programs. Dr. Ucar’s work was also supported by a grant from The Scientific and Technological Research Council of Turkey (TUBITAK). Miss Kim’s research was supported by the NIH Medical Research Scholars Program, a public–private partnership supported jointly by the NIH and generous contributions to the Foundation for the NIH from Pfizer, Inc., Doris Duke Charitable Foundation, Newport Foundation, American Association for Dental Research, Howard Hughes Medical Institute, Colgate-Palmolive Company, as well as other private donors. For a complete list, please visit the Foundation website at: http://fnih.org/work/education-training-0/medical-research-scholars-program.

Additional information

Funding

This study was supported by the NEI and NIAID Intramural Research Programs. Dr. Ucar’s work was also supported by a grant from The Scientific and Technological Research Council of Turkey (TUBITAK). Miss Kim’s research was supported by the NIH Medical Research Scholars Program, a public–private partnership supported jointly by the NIH and generous contributions to the Foundation for the NIH from Pfizer, Inc., Doris Duke Charitable Foundation, Newport Foundation, American Association for Dental Research, Howard Hughes Medical Institute, Colgate-Palmolive Company, as well as other private donors. For a complete list, please visit the Foundation website at: http://fnih.org/work/education-training-0/medical-research-scholars-program.