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Ocular Manifestations in Primary Immunodeficiency Disorders: A Report From the United States Immunodeficiency Network
Michele N Pham1, Ramsay L Fuleihan2, Kathleen E Sullivan3
1Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, Department of Medicine, University of California, San Francisco, San Francisco, Calif.
Background:
Few published studies address eye disease in primary immunodeficiency (PID) despite ocular infections and autoimmune disease being known manifestations of immunodeficient states.
Objective:
Data from the USIDNET Registry provide a resource to study ocular ailments in subjects with PID.
Methods:
Ocular manifestations and patient characteristics were determined using data from 4624 patients with PID enrolled in the US Immunodeficiency Network (USIDNET) Registry.
Results:
A total of 519 (11.2%) patients had recorded ocular diseases. Those with autoinflammatory disorders (n = 4 of 7 [57.1%]), intrinsic and innate immunity defects (n = 9 of 44 [20.5%]), and immune dysregulation (n = 27 of 142 [19.0%]) had the highest percentage of ocular diseases for the PID diagnosis category. Of the 67.6% with infections, 85.5% had conjunctivitis. Bacteria (56.2%) and viruses (27.4%) were the most common microorganisms reported, with Staphylococcus (31.7%), Haemophilus (26.8%), and Streptococcus (22.0%) being the most common bacteria isolated. Those with a history of eye infections had lower immunoglobulin levels, lower CD19 B-cell percentages, and a lower number of protective pneumococcal titers. In patients with noninfectious ocular complications, 30.8% had vision changes, with retina (n = 20 [8.0%]), cataract (n = 16 [6.4%]), and nerve diseases (n = 16 [6.4%]) also being common. Many patients with ocular disease had serious sequelae, with 12.7% undergoing eye surgery and 10.6% having a vision-based disability.
Conclusions:
Vision loss and conjunctivitis were the most commonly reported ocular complications and pose large quality-of-life issues. Learning more about ocular disease in PID will increase awareness about the importance of addressing and evaluating for these ailments.