Primary immunodeficiency disorders linked to ocular manifestations
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Ocular manifestations appeared common among patients with primary immunodeficiency diseases, suggesting a need for more integrated ocular and immunological care, according to a study.
“The eye plays an integral role in our day-to-day life, impacting the way we interact with the world and enjoy and receive life,” Michele N. Pham, MD, clinical assistant professor in the division of pulmonary, critical care, allergy and sleep medicine in the department of medicine at the University of California, San Francisco, told Healio.
“We saw many patients with immunodeficiencies such as common variable immunodeficiency (CVID) coming into clinic with ocular infections and conjunctivitis,” Pham continued. “Because little is known about ocular disease in patients with immunodeficiencies, I wanted to further study this topic.”
For the study, published in The Journal of Allergy and Clinical Immunology: In Practice, Pham and colleagues evaluated data from the United States Immunodeficiency Network (USIDNET) registry.
As of January 2017, there were 4,624 patients with primary immunodeficiency disorder (PID) in the registry, with 11.2% (n = 519) reporting ocular manifestations.
“This is likely underreported given the nature of the database study and the lack of awareness/attention to ocular disease,” Pham said.
Men and boys in the registry had a 1.3 (95% CI, 1.1-1.6) greater risk for ocular disease than women and girls. Patients on immunoglobulin replacement were 2.3 times (95% CI, 1.6-3.4) more likely to have a history of ocular infection as well.
Also, patients with ocular manifestations had an average age of onset of immunodeficiency of 6.9 ± 12.2 years compared with 10.8 ± 16.7 years for patients who did not have any ocular manifestations (P < .001).
Patients with ocular disorders most frequently reported infectious manifestations (64.7%), most commonly conjunctivitis (86.6%), retinitis (2.6%), uncategorized eye infection/erythema (2.6%) and blepharitis (2%).
There also were 209 patients (40.3%) with noninfectious eye disease. The 300 diagnoses in this group included blindness/vision changes (32%), uveitis (7.3%), photophobia (6.7%), retina diseases (6.3%) and cataracts (4.7%).
The researchers further found that autoimmune ocular diseases accounted for 3.4% (46 of 1,348) of the diseases reported as autoimmune, including Sjögren’s syndrome (45.7%; n = 21) and uveitis (30.4%; n = 14).
Although there was no difference in the average IgG levels between patients who did and did not have ocular manifestations, patients with ocular disease had lower IgA (72.2 ±118 mg/dL vs. 74.2 ± 138 mg/dL; P = .01) and IgM (60.5 ± 90.1 mg/dL vs. 72.5 ± 155 mg/dL; P < .001) levels.
There were significantly lower levels of serum immunoglobulins including IgG (550 ± 436.3 mg/dL vs. 638 ± 458.2 mg/dL; P < .001), IgA (56.4 ± 109 mg/dL vs. 74.2 ± 138.4 mg/dL; P < .001) and IgM (49.3 ± 86.2 mg/dL vs. 72.5 ± 155 mg/dL; P < .001) among patients with eye infections as well.
In addition, patients who had ocular manifestations or ocular infections had lower percentages of CD19 and CD20 cells and higher T-cell percentages than those who did not have ocular manifestations or infections.
Among 1,592 patients with CVID, comprising 34.4% of the registry, 11.4% (n = 182) had a history of ocular disease. They had an average age of symptom onset of 15.1 ± 16.1 years, whereas those without ocular disease had an average age of symptom onset of 21.2 ± 19.6 years (P = .002).
By recognizing possible eye diseases, the researchers wrote, doctors may be able to diagnose and treat immunodeficiency syndromes and ocular complications earlier and prevent more serious long-term consequences.
“Learning more about ocular disease in PID will increase awareness about the importance of addressing and evaluating for these ailments. Many of these ocular ailments can be preventable if screened and evaluated for,” Pham said.
The researchers additionally encouraged immunologists to ask their patients about ocular symptoms and consider eye examinations as part of their management strategies. Next, Pham said, she and her colleagues will look at prospective and interventional studies to better quantify occurrence and prevent and treat disease.
For more information:
Michele N. Pham, MD, can be reached at michele.pham@ucsf.edu.