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Introduction to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet´s disease and identify prognostic factors. Methods we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet´s disease and hospitalized in an Internal Medicine Department. Results the mean age of the Behçet´s disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet´s disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence.

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Cardiac involvements in systemic sclerosis have a poor outcome. We report here a case of 28 year-old-man with history of systemic sclerosis developing a fatal myocarditis.Initially patient has disabling joint manifestation. Systemic sclerosis is diagnosed after progressive development of cutaneous, pulmonary, and gastrointestinal manifestations. One year after systemic sclerosis diagnosis, our patient develops heart failure signs when he is already treated with methotrexate (15 mg/week) and coticosteroids (15 mg/day). Echocardiography show global hypokinesia with low left ventricular ejection fraction (32%). Natriuretic peptides and troponin levels are high. Anti-centromere and anti-SRP antibodies are positive. Corticosteroids dose is increased to 1 mg/kg/day, and methotrexate was given 25 mg/week without improvement. Cyclophosphamide was started but patient died within six months. Despite immunosuppressive therapy, myocarditis outcome is poor since patient was in heart failure at presentation, and has anti-SRP antibodies.

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