FRI0500 BEHÇET SYNDROME AND CENTRAL NERVOUS SYSTEM INVOLVEMENT: A DESCRIPTIVE STUDY IN A COHORT OF PATIENTS IN OUR RHEUMATOLOGY DEPARTMENT
Authors: N. Plaza Aulestia, M. J. Pérez, S. Rodríguez Montero, J. L. Marenco
Journal: Annals of the Rheumatic Diseases (2020)

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 aphtosis 33 (100) Genital ulceration 23 (69.69) Skin (Pseudofolliculitis/Erythema Nodosum) 12 (36.36) Ocular 22 (66.66) Anterior Uveitis 8/22 (36.36 ) Posterior uveitis 3/22 (13.63 ) Panuveitis 5/22 (22.72 ) Vitritis 1/22 (4.54 ) Retinal vasculitis 6/22 (27.27 ) Vascular 7 (21.21) Arthralgias/arthritis 20 (60.60) Gastrointestinal 2 (6.06) Central Nervous System 9 (27.27 ) Demyelinating lesions 5/9 (55.55 ) Stroke 2/9 (22.22 ) Epilepsy 1/9 (11.11 ) Vasculitis 1/9 (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 literature 1,2 . 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 atherosclerosis 3 . References: [1] 10.1016/B978-0-7020-4088-7.00110-3 [2]10.1046/j.1365-4362.2003.01741 [3]10.1007/s00415-013-7209-3 Disclosure 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