Anasarca Revealing Severe Cardiac Involvement Due to Behçet's Disease (BD): A Case Report.

Behçet's disease (BD) is a systemic vasculitis which is most often manifested by recurrent oral aphthosis, genital aphthosis, and ocular involvement with sometimes visceral damage, in particular neurological, digestive, vascular, or renal. We report the case of a 21-year-old man admitted for anasarca who revealed severe cardiac involvement associating endomyocardial fibrosis, intracardiac thrombi and involvement of the tricuspid valve in the context of BD diagnosed a posteriori. Cardiac involvement is exceptional during BD, especially as a mode of entry into the disease. It can be particularly severe, hence the need for early diagnosis, rapid and sometimes aggressive management. Close monitoring is also necessary in order to watch for the occurrence of visceral manifestations, particularly in young patients.

[Adult onset Still's disease and lymphoma: a rare association]. / Maladie de Still de l'adulte et lymphome: une association rare.

Adult onset Still's disease (AOSD) and lymphomas are diseases characterized by very similar clinical and histopathological manifestations. The association between these diseases has rarely been reported in the literature. We here report the case of a 26-year old patient diagnosed with adult onset Still's disease. She had been previously treated for large B-cell Non-Hodgkin lymphoma (LBCNHL) with chemotherapy and haemopoietic stem-cell autograft with complete remission. The association between AOSD and lymphoma is rare and in all cases AOSD has been diagnosed before lymphoma. The peculiarity of this study lies in the succession LBCNHL- AOSD and raises several hypotheses on the association between these two diseases. The association between non-autoimmune diseases and lymphoid hemopathies have been widely demonstrated, whether it is the progression of autoimmune diseases vs lymphoma or non-autoimmune disease manifestations occurring in patients with this disease. This study highlights the difficult distinction between these diseases.

[Behcet syndrome: thirty comments with lung and vascular injury of peripheral vessels]. / Maladie de Behçet: trente observations avec atteintes vasculaires pulmonaires et des vaisseaux péripheriques.

INTRODUCTION: Behcet's disease is a systematic vasculitis of unknown cause, characterized essentially by eye, cutaneous, articular, neurological and vascular manifestations. METHODS: We retrospectively analysed the Behcet's disease cases that were followed up in our ward from January 2000 to January 2009. The inclusion criteria were those of International Study Group on Behçet's disease (aphthosis mouth was required). Data were retrieved and analysed with two softwares (Access(®) and Epi Info(®)). RESULTS: We observed 30 cases with vascular lesions on a series of 92 patients with Behcet's disease. Most patients were male, with an average age around 40. The venous manifestations, concerning essentially the lower limbs (deep and superficial thrombosis) were found at 27 patients (90 %), and the average of age during the appearance of the venous lesions was 40 years. Arterial lesions appear more late in 13 patients (43 %) (average of age 43 years). We noted, on the other hand, 11 cases of aneurysms and five cases of arterial thrombosis. The use of corticosteroids was necessary in all cases in association with the others drugs (anticoagulants, colchicine, immunosuppressors). Among the patients having had aneurysms, six were treated surgically. The outcome was favorable for most patients. Two patients had pulmonary embolism and two post-surgery complications. One patient died in the consequences of an intragastric break of an aneurysm of the abdominal aorta. CONCLUSION: The vascular involvement in Behcet's disease is manifested primarily by thrombophlebitis. Achieving blood pressure, less common, is problematic therapeutic because of the recurrent and life threatening.