Uveitis as a Correlate of Systemic Manifestations in Patients Diagnosed with Behçet's Disease

Uveitis is common in Behçet's disease (BD), but risk stratification of potentially severe systemic disease by ocular involvement is still impossible. Here, we aimed to determine whether the risk for systemic manifestations of BD in Patients Diagnosed with Behçet's Disease (PDBD) depended on the presence of intra-ocular inflammation in one of the largest global health research networks.

Screening > 150 million Electronic Medical Records (EMR), we compared subjects whose ICD-10 codes selected them as PDBD with or without a BD uveitis-reconcilable coding pattern. Outcomes included relative risk for systemic BD manifestations after diagnosis, likelihood of inpatient consultations, and probability of systemic manifestations over time.

3474 subjects met inclusion criteria, with 458 in the ocular cohort. Ocular PDBD had more than a two-fold risk of skin and vascular manifestations than non-ocular PDBD, increased risk for musculoskeletal and central nervous system manifestations, was twice as likely to receive inpatient consultations when hospitalized and showed a much shorter median time to extraocular systemic disease onset (413 days) than non-ocular PDBD (1729 days). The risk for genital lesions and the time to initial diagnosis were similar between the groups.

Within the constraints of an EMR-based study, our results indicate a significantly higher risk for extraocular manifestations and an overall more severe disease course for PDBD with uveitis than for those without. Our findings provide rationale for confirmation in cohorts outside of big data and justify an intensified multidisciplinary clinical approach to ocular PDBD to prevent, detect, and treat the associated systemic disease.