Background Behcet's Disease is a rare type of vasculitis that involves both arterial and venous blood vessels of all sizes. The type of organ involvement and overall disease activity evaluated in the clinical practice determine the course of treatment and the decision to initiate immunosuppression. Activity scores such as Birmingham Vasculitis Activity score (BVASv3), Behcet's Disease Current Activity Form2006 (BDCAF), or damage indices like Vasculitis Damage Index (VDI) have been developed in this respect.
Objectives To evaluate the ability of classical immunosuppressant therapy to prevent damage progression. To find the correlation between disease activity scores: BVASv3, BDCAF, long term treatment, immunosuppressant use and damage after remission, as calculated by VDI.
Methods A study on a cohort of patients diagnosed with Behcet's Disease from an Internal Medicine and Rheumatology Clinic was performed. Activity and damage scores,BVASv3,BDCAF and VDI after obtained remission, were calculated. The documented cases were diagnosed according to the International Criteria for Behçet's Disease (ICBD).Windows Excel/SPSS20.0 (Spearman's correlation)were used to analyse the data.
Results The study included 16 patients treated with long term cortisone and immunosuppressive therapy. The mean age at the time of the diagnosis was 32.3years with a male predominance 62% (10 patients). Severe systemic involvement was present in 10 cases (Ophthalmological involvement-6cases, recurrent venous thrombosis-6cases, pulmonary vasculitis-1 case, severe cardiac involvement-1case, central nervous system involvement-3cases) and all patients received classical immunosuppression (cyclophosphamide, azathioprine).The mean scores for BVASv3 and BDCAF at the time of the diagnosis were 9 and 4.12.A strong correlation was identified between BVASv3 and BDCAF (r=0.830, p<0,001). The use of immunosuppressive therapy due to severe organ involvement and long-term immunosuppression correlated stronger with BVASv3 (r=0.718) than with BDCAF (r=0.533). Vasculitis damage index (VDI) calculated after remission was obtained. There was an important correlation between disease activity scores and damage (BVASv3-VDI r=0,687,p<0,001, BDCAF-VDI r=0,676, p<0,001). Types of treatment were evaluated, a comparison was made between long-term cortisone therapy and immunosuppression.There was a stronger correlation between long term cortisone use and VDI (r=0,600) than between immunosuppression duration and damage (r=0,472).
Conclusions Damage progression is influenced by disease activity, as calculated by activity scores (BVASv3 and BDCAF). Classical immunosuppression is used for severe organ involvement and for limiting new organ lesions once started. There was a stronger correlation between long-term cortisone use and VDI than between immunosuppression duration and damage. The damage index increased by irreversible organ damage due to disease activity and long term cortisone use, but not due to the immunosuppressive therapy.
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Acknowledgements The first two authors,Sinziana Daia-Iliescu and Casandra Buzatu contributed equally to this study.
Disclosure of Interest None declared