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Assessment of CTLA-4 Deficiency–Related Autoimmune Choroidopathy Response to Abatacept

Carol L. Shields, MD1; Emil A. T. Say, MD1; Arman Mashayekhi, MD1; Sunir J. Garg, MD2; James P. Dunn, MD2; Jerry A. Shields, MD1
[+] Author Affiliations
1Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
2Uveitis Unit of the Retina Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
JAMA Ophthalmol. 2016;134(7):844-846. doi:10.1001/jamaophthalmol.2016.1013.
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This study describes a patient with common variable immune deficiency who was later identified as having CTLA-4 deficiency and visually disabling autoimmune choroiditis that responded dramatically to abatacept, a CTLA-4 analogue.

Over the years, patients with recurrent infections and hypogammaglobulinemia generally have been classified as having common variable immune deficiency (CVID), without knowledge of the exact immune pathway defect. Cytotoxic T-lymphocyte antigen 4 (CTLA-4) is a recently described protein that acts as a barrier to T-cell activation and is an important immune modulator.15 The loss of this protein in mice causes fatal autoimmune disease.4 Several investigators observed that CTLA-4 operates as an early checkpoint blockade of T-cell response to antigens and that this regulation is critical for prevention of autoimmunity.15 The presence of CTLA-4 averts immune targeting of host tissues, whereas a deficiency results in autoimmunity.25 Herein, we describe a patient with CVID who was later identified as having CTLA-4 deficiency and visually disabling autoimmune choroiditis that responded to abatacept, a CTLA-4 analogue. The requirement for approval of the study was waived by the Wills Eye Hospital Institutional Review Board, and the patient provided written consent to allow her data to be used in research.

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Figure 1.
Autoimmune Choroidopathy From CTLA-4 Defiiciency

A-D, CTLA-4–deficient autoimmune choroidopathy is seen on ophthalmoscopy. E and F, Findings on optical coherence tomography are shown.

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Figure 2.
Prompt Resolution of the Autoimmune Infiltration and Detachment With Abatacept

Shown are the findings after 3 months of abatacept therapy, a CTLA-4 therapy. A, Resolution of choroidal infiltration, with chorioretinal scar at site of needle biopsy in the right eye. B. Focal choroidal inflammatory sites were less apparent in the left eye. C. Resolution of subretinal fluid in the right eye with residual subretinal precipitates. D. Normal findings in the left eye.

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