Thu0554 the Clinical Utility of Two Vasculitis Activity Scores(bvas and Bdcaf) in Behçet’s Syndrome:a Prospective Cohort Study

Abstract

Background:Behçet’s Disease(BD) is a rare chronic autoinflammatory condition that can lead to irreversible organ damage. The potential for multi-organ involvement and fluctuating activity highlights the need to perform a careful and systematic assessment of disease activity that is sensitive to change. Several disease activity tools have been used in both daily practice and clinical trials, yet there is no published data comparing the clinical utility of different tools in informing changes to therapy.Objectives:To compare the utility of two major activity scores:BD Current Activity Form (BDCAF2006)1 and Birmingham Vasculitis Activity Score (BVAS)2 in predicting physician’s decision to adjust treatment (step-up/step-down) in patients with BD.Methods:A 6-month prospective observational study was performed in a cohort of patients meeting the International Criteria for Behcet’s Disease(ICBD),at the National Centre for BD in Liverpool,UK. Participants were described for their demographics, clinical manifestations and treatment plan.BVAS and BDCAF2006 activity scores were completed for each patient at evaluation.The outcome of interest was treatment change which was classified as ‘step-up’ or ‘step-down’, reflecting escalation or de-escalation in treatment (dosage adjustment or adding new immunosuppressant), respectively. We assessed the association between BVAS and BDCAF scores and step-up/step-down treatment using Spearman rank correlation and multivariate logistic regressions, adjusting for gender, age and patient’s perception of disease activity on visual analogue scale(VAS).Odds ratios(OR) and 95% confidence intervals were calculated. Data analysis was conducted in Microsoft Excel,SPSS 2.0 and STATA.Results:Ninety-five patients met inclusion criteria: 25 males(26.3%) and 70females(73.7%) with a mean age at diagnosis of 32.7years(±11.3 SD). HLAB51 was positive in 11/51 cases(11.6%).The most frequent clinical manifestations were oral ulcerations(100%), genital ulcerations(94.7%) followed by papulo-pustular skin lesions(37.8%) arthralgia(31.6%)and headache(30.5%).Mean BVAS score(range 0-6) was 2.14(±1.8 SD) and mean BDCAF score (range 0-8) was 3.04(±1.72 SD). Both BVAS and BDCAF correlated with decision to step-up treatment (r=0.752; r=0.370, respectively).Furthermore, BVAS was more strongly associated with decision to step-up treatment than BDCAF(OR 4.25 95%CI 2.37 to 7.61; 1.51 95%CI 1.15 to 2.00, respectively). Adjusting for gender, a stronger association was observed in male participants across BVAS and BDCAF scores(OR 5.89 95%CI 1.17 to 29.63; 3.48 95%CI 1.20 to10.09,respectively). Following adjustment for patient’s perception of their disease (VAS), BVAS remained significantly associated with treatment step-up(OR 3.87 95%CI 2.08 to7.19) but not BDCAF(OR 1.30 95%CI 0.91 to1.84).Regarding different clinical manifestations,the BVAS mucocutaneous and ocular activity showed a significant odds ratio for step-up therapy(OR=5.78, CI:1.49-22.15; and OR=4.2,CI: 2.26-7.83).Conclusion:BVAS can be a useful tool to asses BD ...

Ultrasonographic measurement of the vascular wall thickness and intima-media thickness in patients with Behçet’s disease with symptoms or signs of vascular involvement: A cross-sectional study

“…This may be related to the fact that the scales examine other organ involvements rather than vascular activation. In a study by Buzatu et al,[ 26 ] it was stated that the Birmingham Vasculitis Activity Score, which evaluates vascular activation in BD patients, was more sensitive than BDCAF. Therefore, not evaluating vascular activation with a scale that evaluates vascular activation in BD patients is a limitation of our study.…”