AB0604 CLINICAL FEATURES AND TREATMENT IN A COHORT OF PATIENTS WITH BEHÇET DISEASE IN A TERTIARY HOPITAL OF BARCELONA
Authors: Joan Maria Mercadé Torras, Alfredo Guillén-Del-Castillo, Segundo Bujan Rivas, Jaume Mestre Torres, Roser Solans-Laqué
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Behçet disease (BD) is considered a systemic vasculitis according to the Chapel-Hill classification, which occurs most frequently between latitudes 30 and 45° north and in the area of the Old Silk Route. Turkey has the highest prevalence, followed by Japan. In Spain, a prevalence of 7.5 cases per 100000 persons has been estimated. The epidemiological and clinical characteristics of European patients with BD vary with respect to those of the Turkish and Japanese cohorts. <h3>Objectives:</h3> Description of clinical features and treatment received in a cohort of patients diagnosed with BD in an internal Medicine Unit of a tertiary centre from Barcelona. <h3>Methods:</h3> Retrospective, observational study. Epidemiological, clinical and laboratory data were obtained from clinical charts. SSPS package was used to perform statistical analysis. <h3>Results:</h3> 132 patients (56.6% men) diagnosed over the last 30 years and followed-up until the censoring data were included. 112 (84.8%) were Caucasians, 15 (11.4%) from North africa, 4 (3%) asiatic and 1 (0.8%) from South america. 2 (1.5%) patients had a family history of BD and 9 patients (6.8%) a family history of other rheumatic diseases. 43.9% (58 patients) were HLA-B51. Oral or genital ulcers were present in 131 (99.2%) patients and skin involvement in 106 (80%) cases, 44.6% had erythema nodosum, 59% had acne-like lesions and 9% had cutaneous vasculitis. Ocular involvement was observed in 69 cases (52.3%): 27 patients unilateral and 8 bilateral anterior uveitis; 14 unilateral and 3 bilateral posterior uveitis; 10 patients unilateral and 14 bilateral panuveitis; 19 unilateral retinal vasculitis and 12 bilateral. Neurological involvement was present in 37 (28%) patients: 10 parenchimal disease and 17 cases non-parenchymal disease (aseptic meningitis or vasculitis); 8 patients benign intracranial hypertension, and 3 had dural sinus thrombosis. Articular involvement was recorded in 79 (59.8%) patients (it was observed 25 monoarthritis, 24 oligoarthritis, 6 poliarthritis and 58 patients had arthralgia). Vascular involvement was present in 43 (32.6%) cases: deep venous thrombosis in 38 patients (11 cases in locations other than the extremities); pulmonary embolism in 7; 21 thrombophlebitis; and 6 patients aneurysms (only 2 pulmonary arterial aneurysms). Digestive involvement was present in 12 (9,1%) patients, with predominant colon involvement (8 cases). The most prescribed drugs were corticosteroids (85.6%) and colchicine (77.3%), followed by azathioprine (36.4%) and cyclosporine a (33.3%). Other prescribed drugs were thalidomide (6.1%), chlorambucil (6.9%), methotrexate (4.5%), anti-TNF-alpha therapies (infliximab 6.8% and adalimumab 2.3%), cyclophosphamide (3%), mycophenolate (3.8%), leflunomide (1.5%) and 22% received anticoagulation. <h3>Conclusion:</h3> Clinical features of our patients are similar to those of other European cohorts, although a high prevalence of organic involvement (ocular, neurological, vascular and joint) should be highlighted. <h3>References</h3> [1] Saadoun D, Wechsler B. Behçet's disease. Orphanet J Rare Dis. 2012Apr12;7;20. doi: 10.1186/1750-1172-7-20. [2] Mendes D, et al. Behçet's disease – a contemporary rewiew. J autoimmun. 2009May-Jun;32 (3-4): 178-88. doi: 10.1016/j.jaut.2009.02.011. [3] Leccese P, et al. Behçet's síndrome in nonendemic regions. Curr Opin Rheumatol. 2017. doi: 10.1097/BOR.0000000000000349 <h3>Disclosure of interests:</h3> None declared