The Risk of Developing Ocular Involvement Among Behçet's Disease Patients Presenting with Mucocutaneous Involvement at Disease Onset: Data from the International AIDA Network Behçet's Disease Registry

Introduction: Behçet's disease (BD) frequently arises with exclusively mucocutaneous involvement, but some patients will develop major organ involvement, including ocular inflammation. This study aims to assess the patients' demographic and clinical characteristics that may be associated with the development of ocular involvement in patients with BD with exclusively mucocutaneous involvement in the early stages. Methods: Patients' data were collected in the International AutoInflammatory Disease Alliance (AIDA) Network registry dedicated to BD. Results: A total of 328 patients with BD were enrolled, 36 (11%) of whom developed ocular involvement over time. The following variables were significantly associated with the development of ocular inflammation at binary logistic regression: positive family history for BD (OR 4.32, 95% CI 1.16-14.72, p = 0.02), Arab ethnicity (OR 3.6, 95% CI 1.3-9.9, p = 0.01), presence of major oral aphthous ulceration (OR 3.26, 95% CI 1.18-8.99, p = 0.02), pseudofolliculitis plus erythema nodosum (OR 4.58, 95% CI 1.33-15.7, p = 0.016); conversely, white patients/patients of European descent (OR 0.33, 95% CI 0.12-0.91, p = 0.03) and the presence of a low number (one to two) ulcers at genital aphthous flares (OR 0.002, 95% CI ~ 0-0.07, p = 0.0006) were protective against the occurrence of ocular inflammatory manifestations. Genital aphthosis with more than five concurrent ulcers was associated with eye involvement in Arab patients (OR 209.2, 95% CI 5.8-7497, p = 0.003). Conclusions: Major oral aphthosis, genital aphthous attacks with more than five concurrent ulcers, erythema nodosum coexisting with pseudofolliculitis, a positive family history of BD, and Arab ethnicity are associated with a higher risk of ocular involvement when BD arises with the sole mucocutaneous involvement.