BackgroundValidated disease activity scores and damage measurements were developed over time in order to allow a better way to evaluate patients and decide treatment plans.There are scores designed for a great variety of vasculitis like Birmingham Activity Score and others that are more specific like Behcet's Disease Current Activity Form2006.ObjectivesTo evaluate the ability of the activity scores (BVASv3and BDCAF)to predict damage,and the influence of immunosuppressive therapy on damage progression,as measured byVDI, in a group of patients with Behcet's Disease.MethodsA study was performed on a cohort of patients diagnosed with Behcet's Disease under surveillance in one tertiary Rheumatology Centre, from a non-endemic area.All documented cases of Behcet's Disease have been diagnosed according to The International Criteria for Behçet's Disease.The Birmingham Activity Score (BVAS)v3, Behcet's Disease Current Activity Form2006 (BDCAF)and Vasculitis Damage index (VDI) were calculated for all patients. Spearman's correlation coefficients were calculated between BVASv3 Score, BDCAF, VDI and immunosuppressive treatment.WindowsExcel/SPSS20.0 has been used to analyse the data.Results20 patients were included in the study, with ages at the time of the diagnosis between 13and 60years, 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. In the clinical case series, Spearman's rank correlation coefficient between BVASv3 and BDCAFwas strong r=0,862with p<0,001.The outcome analysis after remission was calculated and rank correlation coefficient betweenVDI, 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). Since the cause of damage (vasculitis vs. treatment)is not taken into consideration when we calculate VDI, we tried to observe if there are any connections between this and immunosuppression duration. There was a 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).ConclusionsBirmingham Vasculitis Activity score (BVAS) v3 and Behcet's Disease Current Activity Form2006 (BDCAF) are reliable tools for evaluating disease activity in patients with Behcet's Disease. They are able to anticipate the need for immunosuppressive therapy and the damage progression, as calculated with Vasculitis Damage Index (VDI).References Mukhtyar C, Lee R, et al. Modification and validation of the Birmingham vasculitis activity score, Ann Rheum Dis, 2009.Flossmann O, et al. Development of comprehensive disease assessment in systemic...
characteristics of autoimmune diseases associated with sarcoidosis (sarcoidosisoverlap group) patients and isolated sarcoidosis (isolated sarcoidosis group) were analyzed and compared. Results: Autoimmune disease was detected in 15 (11.5%) of 131 patients with sarcoidosis (1Sjögren syndrome, 3rheumatoid arthritis, 1Still disease, 1scleroderma, 4ankylosing spondylitis, 1familial Mediterranean fever, 1gut arthritis, 1immune trombocytopenic purpura, 1Hashimoto thyroiditis and 1Graves disease). Most of these diseases occurred before (such as RA, AS, Still, FMF) and others after sarcoidosis diagnosis. Among 15 sarcoidosis patients with autoimmune disease 10 were female and 5 were male, the mean age was 50.8 years and mean disease duration was 3 months (1-30 months). When compared with isolated sarcoidosis patients, more hand finger joint involvement, RF positivity, higher ESR and less NSAIDs usage were found in patients with sarcoidosis-overlap group (p=0.035, p=0.049, p=0.015, p=0.018 respectively). There was no statistically significant differences between the two groups when evaluated for demographic, clinical parameters and other treatment modalities. Conclusions: Concomitant autoimmune diseases in patients with sarcoidosis may be often seen. This patients are characterized with more hand finger joint involvement, RF positivity, higher ESR and less NSAIDs usage. Therefore, in patients with a diagnosis of sarcoidosis, it is necessary for the physician to be careful and to make a wider differential diagnosis in terms of the presence of another underlying autoimmune disease. Multicenter, prospective studies involving large numbers of patients are needed to understand whether the association of sarcoidosis-autoimmune diseases is based only on coincidence or on a common etiopathogenesis.
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