Background:Behçet’s disease (BD) is a variable vessel vasculitis characterized by mucocutaneous lesions (oral and genital ulcers). In addition, it can affect other systems such as vascular, ophthalmological, gastrointestinal tract or central nervous system (CNS). The latter being one of the most serious manifestations of Behçet’s disease. We present a descriptive observational study of a cohort of patients diagnosed with Behçet’s disease with CNS involvement.Objectives:Our objective is to describe the prevalence and characteristics of various clinical manifestations in patients with Behçet.Methods:Observational, descriptive, cross-sectional, retrospective design performed in patients with Behçet’s disease in follow-up by the Rheumatology department of the Hospital de Valme, until October 2019. The following information is collected from medical records; sex, mean age at diagnosis, clinical characteristics, HLA B51 positivity and treatment.Results:Thirty-three patients were diagnosed with Behçet’s disease, with a mean age at diagnosis of 36.22 +/- 10.94 years, female predominance 19 (57.57%). Regarding the determination of HLA B 51; 5 (15.15%) positive, 6 (18.18%) negative and 22 (66.66%) not determined. The following information about clinical manifestations is collected:Clinical manifestations n (%)Oral aphtosis33 (100)Genital ulceration23 (69.69)Skin (Pseudofolliculitis/Erythema Nodosum)12 (36.36)Ocular22 (66.66)Anterior Uveitis8/22 (36.36)Posterior uveitis3/22 (13.63)Panuveitis5/22 (22.72)Vitritis1/22 (4.54)Retinal vasculitis6/22 (27.27)Vascular7 (21.21)Arthralgias/arthritis20 (60.60)Gastrointestinal2 (6.06)Central Nervous System9 (27.27)Demyelinating lesions5/9 (55.55)Stroke2/9 (22.22)Epilepsy1/9 (11.11)Vasculitis1/9 (11.11)We identified 9 patients with CNS involvement; 5 demyelinating lesions (55%), 2 strokes (22%), 1 epilepsy/seizure (11%) and 1 case of CNS vasculitis (11%). Fifty-five percent of the patients were female (55%). Two patients were positive for HLA B51 (22%) and the rest had not been determined. Regarding treatment, all patients received both, oral and intravenous, corticosteroids (prednisone, methylprednisolone) and seven of them received DMARDs; 2 methotrexate (28%), 2 cyclosporine (28%), 1 sulfasalazine (14%), 1 azathioprine (14%) and 1 mercaptopurine (14%, due to intestinal involvement). Two of them needed biologic DMARD with TNF inhibitor agents (Infliximab and Adalimumab).Conclusion:Although neurological involvement is not common in BD, we describe higher prevalence (22%) than other series in the literature1,2. Given the heterogeneity of the clinical presentation there is a wide differential clinical diagnosis to rule out; demyelinating diseases (multiple sclerosis), such as demyelinating lesions presented in 5 of our patients (55%), infectious diseases (tuberculosis), other autoimmune diseases (Vogt–Koyanagi–Harada Syndrome, Eales Sd) or ischemic stroke due to atherosclerosis3.References:[1] 10.1016/B978-0-7020-4088-7.00110-3[2]10.1046/j.1365-4362.2003.01741[3]10.1007/s00415-013-7209-3Disclosure of Interests:NAHIA PLAZA AULESTIA: None declared, MARÍA JOSÉ PÉREZ: None declared, Sergio Rodríguez Montero: None declared, Jose Luis Marenco Speakers bureau: ABbvie, Pfzer, lilly
Anti-endothelial cell antibodies and central nervous system involvement in Behçet's disease