INTRODUCTION: Behçet's disease (BD) is a multi-system, chronic and relapsing disorder classified as "vasculitic syndrome". It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet's disease. METHODS: The authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet's disease. RESULTS: A 19-year-old boy was admitted to our hospital for the investigation of "fever of unknown origin", weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as "an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells". Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet's disease. The medical management included immunosuppressant and anticoagulation. CONCLUSION: Behçet's disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.
INTRODUCTION: Behçet's disease (BD) is a multi-system, chronic and relapsing disorder classified as "vasculitic syndrome". It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet's disease. METHODS: The authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet's disease. RESULTS: A 19-year-old boy was admitted to our hospital for the investigation of "fever of unknown origin", weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as "an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells". Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet's disease. The medical management included immunosuppressant and anticoagulation. CONCLUSION: Behçet's disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.
Entities:
Keywords:
B51(5); Behçet’s disease; HLA-B; Right ventricular mass; Scrotal ulcer
Authors: G Hatemi; A Silman; D Bang; B Bodaghi; A M Chamberlain; A Gul; M H Houman; I Kötter; I Olivieri; C Salvarani; P P Sfikakis; A Siva; M R Stanford; N Stübiger; S Yurdakul; H Yazici Journal: Ann Rheum Dis Date: 2008-01-31 Impact factor: 19.103
Authors: Bülent B Altunkeser; Niyazi Görmüş; Kurtuluş Ozdemir; Kadir Durgut; Ayşe Ozdemir; Hasan Solak; Hasan Gök Journal: Heart Vessels Date: 2002-07 Impact factor: 2.037
Authors: Alexandra Briosa; Ana Catarina Gomes; Ana CastelBranco; Margarida Cunha; Sandra Sousa; Ana Rita Almeida; Paulo Calhau; Hélder Pereira Journal: Eur Heart J Case Rep Date: 2021-10-08