Association between ocular manifestations and incident cardiovascular comorbidities in Ehlers Danlos Syndrome

To determine if ocular manifestations in Ehlers-Danlos Syndrome (EDS) are associated with increased risk for cardiovascular (CV) comorbidities.

Retrospective cohort study using a US database platform that aggregates de-identified health records. The study group included EDS patients with at least 1/8 ocular manifestations and the control group included those without ocular manifestations. Groups were propensity score matched (PSM) for age at diagnosis, sex, race, and ethnicity. The primary outcome was the relative risk (RR) of a subsequent encounter diagnosis for 1/10 CV comorbidities. The secondary outcome evaluated the relationship between specific ocular diagnoses and specific incident CV comorbidities. Tertiary analysis compared healthcare utilisation between groups. Successful PSM was determined as a standard mean difference <0.1, and statistical significance included 95% confidence intervals (CI) and RR < 0.9 or >1.1. Analyses were performed on the platform and RStudio.

After PSM, 13,197 predominantly White female EDS patients were included in each group. EDS patients with ocular manifestations were at significantly increased risk for 70% of included CV outcomes and at significantly increased risk for combined CV outcomes (RR 1.24, 95% CI 1.11-1.31). Myopia, idiopathic intracranial hypertension, and dry eye disease conferred the highest RR. Highest risk outcomes included cerebral, coronary, and intracranial arterial events. Lastly, the study group demonstrated significantly higher hospitalisations (1.44, 1.33-1.55), emergency department visits (1.22, 1.13-1.32), and surgeries (1.14, 1.09-1.2).

Ocular manifestations in EDS were associated with a higher incidence of subsequent cardiovascular diagnoses, identifying a potential high-risk subgroup for further investigation.