Risk of Ocular Comorbidities in Patients With Inherited Retinal Diseases

BACKGROUND AND OBJECTIVE: Given limited inherited retinal disease (IRD) treatment options, identifying and treating co-occurring ocular conditions is essential for optimizing outcomes. This study aimed to evaluate the prevalence and types of ophthalmic conditions occurring more frequently in patients with IRDs compared to individuals in the general population, in order to guide screening and management strategies. PATIENTS AND METHODS: Retrospective cohort study utilizing a large International Classification of Diseases, Tenth Revision (ICD-10) coded platform. Patients with ≥ 2 IRD ICD-10 codes were compared to controls with a documented eye exam (1:1) propensity matched on demographics and systemic conditions. RESULTS: At 3 years, IRD patients demonstrated higher risk of several anterior segment abnormalities including cataracts (risk ratio [RR] 1.31, 95% CI 1.27-1.34) and lens dislocation (RR 2.61, 1.73-3.95), and posterior segment conditions including retinal detachment (RR 2.59, 2.31-2.90), retinoschisis (RR 6.85, 4.78-9.82), and cystoid macular edema (RR 6.51, 5.56-7.62). Nystagmus (RR 9.22, 7.38-11.53), color blindness (RR 13.29, 7.72-22.86), and legal blindness (RR 52.15, 35.40-76.85) were also elevated. Five- and seven-year analyses confirmed similar trends. CONCLUSION: IRD patients have a higher risk of several ocular comorbidities, underscoring the need for close monitoring and periodic examinations.