# Real-World Comparison of Overall Survival Among Patients With and Without Inherited Retinal Diseases

## TL;DR

This study found that people under 65 with inherited retinal diseases in the US have a higher risk of death and more health issues compared to those without these diseases.

## Contribution

The study provides real-world evidence of increased mortality and comorbidities in patients with inherited retinal diseases.

## Key findings

- Patients with IRD had a 24% higher risk of death compared to those without IRD.
- IRD patients were at significantly higher risk for physical comorbidities and mental health conditions.
- The study highlights the need for novel therapies to address the clinical burden of IRD.

## Abstract

This study compared real-world overall survival and the risk of physical comorbidities and mental health conditions among patients aged <65 years with versus without inherited retinal diseases (IRDs) in the United States (US). Optum® Electronic Health Record data (January 2014–January 2023) were evaluated for IRD (patients with ≥2 medical visits with an IRD diagnosis; index date: second such medical visit) and non-IRD (patients without an IRD diagnosis; index date: random medical visit) cohorts. Baseline demographics were balanced between cohorts using propensity score matching (2:1). Outcome measures were overall survival (date of death due to any cause) and presence of physical comorbidities and mental health conditions (medical visit with a corresponding diagnosis code). In total, 4594 patients with IRD were matched to 9188 patients without IRD (mean age: 38.7 vs. 38.2 years, 53.9% vs. 55.1% female, mean follow-up: 53.1 vs. 52.8 months). Over 84 months, patients with versus without IRD had a 24% higher risk of death (overall survival: 95.8% vs. 96.7%; hazard ratio: 1.24; 95% confidence interval: 1.00–1.53; p = 0.046) and were at significantly higher risk for each evaluated physical comorbidity and mental health condition (all p < 0.05). The development of novel therapies is thus needed to address the clinical burden of IRD.