| Abstrakt: |
The diagnostic utility of HLA B51 (Human Leukocyte Antigen) in association with Behçet's disease(BD) has been identified. However, the clinical manifestations of HLA B51 positive patients who have not satisfied the criteria for Behcet's have been rarely reported. Our objective was to study the clinical profile of HLA B51 positive patients and to include them under the spectrum of International criteria for Behcet's disease (ICBD) and the modified Japanese criteria, and also to study the outcome in these patients. This is a retrospective prospective observational study conducted over 3 years (2018-2021) in HLA B51 positive patients. The clinical presentations, treatment given and follow up response to treatment were observed. In the study cohort of 28 HLA-B51 positive patients, mean age was 41.7 years. 17 (60.7%) had oral ulcers, 5 (17.8%) genital ulcers, 7 (25%) skin lesions, 11 (39.2%) had Pathergy positivity, 19 (67.8%) ocular, 7 (25%) neurological, 3 (10.7%) Gastrointestinal (GI) and 3(10.7%) had vascular manifestations.12 (42.8%) satisfied ICBD criteria and 2 (7%) satisfied Japanese criteria for BD. 15 (53.5%) were given immunosuppressant therapy with [Azathioprine (AZA) in 10(35.7%), Mycophenolate mofetil (MMF) in 4(14.2%) and cyclophosphamide (CYC) in 1(3.5%)]. Biologics were given in 7 (25%) with [Adalimumab(ADA) in 6(21.4%) and Rituximab (RTX) in 1(3.5%)]. Our study showed female predominance, with more ocular manifestations, oral aphthous ulcers and less gastrointestinal involvement. Azathioprine (AZA) showed better response compared to Mycophenolate mofetil (MMF). [ABSTRACT FROM AUTHOR] |
