Mediastinal fibrosis combined with Behcet's disease. Three case reports.

Purpose. - Intrathoracic manifestations other then vascular aneurysm and th rombosis are rare in the course of Behcet's disease. Mediastinal fibrosis i s also unusual. Methods. - Three cases of Behcet's disease with initial mediastinal fibrosi s are reported Diagnosis means, pathogenic mechanisms and therapeutic modal ities are also more precisely defined. Results. - Three male patients (21, 25 and 27 years of age, respectively) w ere hospitalized for progressive vena cava superior syndrome accompanied by mediastinal fibrosis demonstrated by MRI and/or CT scan which both showed infiltration of the mediastinal fat. Multiple calcifications were also pres ent in one patient. Histology was performed in one case. Thrombosis of the vena cava superior and innominata veins was associated with fibrosis in all three cases. Thrombosis of venae subclavia and left jugularis was also pre sent in one case. Other manifestations leading to the diagnosis of Behcet's disease were demonstrated by the patient's history and, in one case, occur red on the fourth day of hospitalization. After initiation of a treatment i ncluding corticoids, anticoagulants and colchicine, the outcome was favorab le in two cases. The third patient died. Conclusion. - Mediastinal fibrosis combined with Behcet's disease has rarel y been described. However, it should be included in manifestations of the d isease. The causative process which might be vasculitis has to be demonstra ted. (C) 2000 Editions scientifiques et medicales Elsevier SAS.