Applying Vasculitis Activity and Damage Scores in a Group of Patients With Behçet’s Disease – Clinical Utility

Abstract

Background. Behçet’s disease is a rare type of vasculitis. Validated activity and damage scores were developed for vasculitis patients in order to allow a better way to evaluate disease activity and decide treatment plans. Objective. The main objective was to compare two vasculitis activity scores applied to a group of patients diagnosed with Behçet’s disease and establish correlations between them, damage and the need for immunosuppressive therapy. The secondary objective was to evaluate the connection between damage progression, classical immunosuppressant therapy and long-term cortisone use. Methods. A study was performed on a cohort of patients diagnosed with Behçet’s disease according The International Criteria for Behçet‘s Disease (ICBD) under surveillance in one Rheumatology Centre, from a non-endemic area. Vasculitis activity and damage scores were calculated for each patient. Results. 20 patients were included in the study, with a mean age of 35.7 years ± 10.5 years standard deviation(SD), 14 (70%) under the age of 40, with a male predominance 60% (12 patients). All patients presented active disease at the time of the diagnosis. Spearman’s rank correlation coefficient between BVAS v3 and BDCAF was strong r = 0.862 and statistically significant p < 0.001. The outcome analysis after remission was calculated and rank correlation coefficient between VDI, and both BVASv3 and BDCAF was moderate (VDI-BVASv3 r = 0.747, p < 0.001, VDI - BDCAF r = 0.795, p < 0.001). As for immunosuppression induction decision and activity scores, the correlation coefficient was moderate (r = 0.734 for BVASv3, r = 0.647 for BDCAF) with p < 0.001. There was a moderate correlation between immunosuppressive treatment and VDI (r = 0.700, p < 0.001). The cause of damage (i.e. vasculitis vs. treatment) is not taken into consideration when we calculate VDI. Data analysis showed the presence of mild correlation and no statistical impact between cyclophosphamide treatment duration and damage calculated as VDI (r = 0.474, p = 0.36). In contrast, when rank correlation coefficient between cortisone therapy and VDI was calculated, a moderate statistical impact was observed (r = 0.609, p < 0.001). Conclusions. An objective assessment of disease’s activity can be obtained using disease activity indexes. A moderate to strong correlation was obtained between activity indexes, immunosuppressive treatment initiation and damage progression. Comparing the two activity indexes, it resulted that: BVASv3 correlates stronger with the need for immunosuppressive treatment and both of them are equally able to anticipate damage. Damage progression was correlated stronger with long-term cortisone use, rather than immunosuppressive therapy.