‘Vasculo’ & ‘Oculo/Neuro’ Behçet’s Disease: Two Opposing Sides of the Same Coin with Possible Different Pathogenesis

Introduction: Prognosis of vasculo-Behçet’s disease (VBD) is not consistent. Patients exhibiting arterial and central large venous involvement have the poorest prognosis. Given the significance of risk stratification in the management of cardiovascular diseases and the lack of specific diagnostic tests for BD, which depends entirely on clinical manifestations, this study aimed to identify clinical features linked to high-risk VBD for early management and enhanced outcomes. Patients and Methods: Clinical characteristics of 230 BD patients; 96 VBD and 134 non-VBD; were documented and analysed. For risk stratification, VBD were categorised into two clinical groups. Group (A): 37 patients with isolated arterial and/or central large venous involvement(s) who were identified as having high-risk VBD (Hr-VBD), and Group (B) [low risk VBD (Lr-VBD)] comprised 59 patients with isolated peripheral limb deep venous thrombosis. Results: Retinal vasculitis and neuro-BD (NBD) were less frequent in Hr-VBD patients ( P = .03, .06, respectively). However, in multivariate analysis, only NBD was found to be less associated with Hr-VBD ( P = .08). A comparison of VBD and non-VBD validated the inverse relationship between retinal and peripheral vasculitis in both univariate ( P = .01) and multivariate ( P = .049) analyses. Moreover, anterior, posterior, and pan-uveitis were all significantly lower in VBD in univariate ( P = .01, <.001 and .03) and multivariate (.03, .001 and .045) examination. Furthermore, male gender ( P = .01) and disease duration ( P = .045) were associated with VBD. Conclusion: Ocular and NBD are less associated with VBD, and when they co-occur, VBD tends to be of low risk with consequent better prognosis. BD seems to have two distinct opposing pathologies: central ‘oculo and NBD’ and peripheral vasculitis.