Objectives: To describe the clinical manifestations and treatment of flares during follow-up in patients with Behçet’s disease (BD) included in the REGEB cohort over the last decade.

Methods: The Spanish Registry of BD or REGEB (REGistro de la Enfermedad de Behçet as Spanish nomenclature) Project Group was created by the Spanish Internal Medicine Society in 2009 with the aim of compiling a large cohort of Spanish patients with this rare disorder. By July 2012, REGEB has collected 635 BD patients with a multicentre, longitudinal and consecutive design. Diagnosis of BD was performed on the basis of the International Study Group criteria for BD. Since inclusion in the registry, patients have been followed prospectively and new flares have been recorded, defined as those clinical manifestations which have required initiation or modification of immunosuppressive treatment, or prednisone dose at or higher than 10mg/d of prednisone during more than 1 month.

Results: A prospective follow-up of 326 patients included in the registry has been analysed. There are 182 women (56%) and their average age (SD) at the time of diagnosis was 33 (13) years. The majority of them were Caucasian (91%). The median follow-up time from BD diagnosis was 180 months, and the prospective follow-up period from the inclusion in the registry was 80 months. The cumulative clinical manifestations until the initial registration were oral ulcers in 100% of patients, genital ulcers in 221 (68%), arthritis in 147 (45%), erythema nodosum in 96 (29%), fever in 82 (25%), thrombosis in 74 (23%), anterior uveitis in 76 (23%), retinal vasculitis in 46 (14%), posterior uveitis in 35 (11%), aseptic meningitis in 32 (10%), and other neurological manifestations in 15 (5%) patients. One hundred and fifty-six (48%) patients received immunosuppressants and 47 (14%) biological therapy.

During follow up (period from the inclusion in the REGEB to the last visit) 68 (23%) patients presented at least a severe outbreak. The median time from the BD diagnosis to the first flare were 170 months. The main clinical manifestations were oral-genital ulcers in 43 (63%) patients, uveitis in 31 (45%), arthritis in 13 (19%), neurological in 16 (24%), vascular in 10 (15%), and gastrointestinal in 3 (4%) patients. Immunosuppressants were used in 37 (54%) patients and biological therapy in 14 (21%). Biological therapy was mostly used due to refractory disease, the majority of cases because ocular manifestations. There were no differences in terms of age, sex, race and previous clinical manifestations between patients who suffered from flares and those who did not. Flares were more frequent in patients who have received previous immunosuppressive or biological treatment (35% vs 13% and 48% vs 19%, respectively) (p<0.001 in both cases), probably reflecting a more severe disease.

Conclusion: The long-term follow-up of BD patients from REGEB cohort showed that 10 years after diagnosis, a fifth of them may continue to present severe flares requiring systemic treatment. The use of biological therapy increased over time and their main indication was refractory disease.

Disclosure of Interests: Mónica Rodríguez Carballeira: None declared, Roser Solans: None declared, Raquel Ríos Fernández: None declared, Begoña Escalante: None declared, Brenda Maure: None declared, Alejandra Fernández: None declared, Robert Hurtado: None declared, Rafael Boldova: None declared, Gerard Espinosa Speakers bureau: Glaxo-Smith-Kline, Janssen, Boehringer, Rovi