Altun Ibrahim, Guz Goksel, Akin Fatih, Kose Nuri, Altun Ilknur, Gunaydin Zeki Yuksel, Sahin Cem
Mugla Sitki Kocman University, Faculty of Medicine, Department of Cardiology, Mugla, Turkey.
Istanbul University, Faculty of Medicine, Department of Cardiology, Istanbul, Turkey.
Int Cardiovasc Res J. 2014 Dec;8(4):178-80. Epub 2014 Dec 1.
Multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems. Definitive diagnosis can only be made based on biopsy findings. In this case, the biopsy specimen of the patient demonstrates pulmonary hyalinated granuloma or sclerosing mediastinitis. There are few reports of multiple fibrosclerosis with heart failure. Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy. Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.
多灶性纤维硬化是一种病因不明的罕见综合征，其特征是纤维化累及多个器官系统。明确诊断只能基于活检结果。在本病例中，患者的活检标本显示为肺透明变性肉芽肿或硬化性纵隔炎。关于多灶性纤维硬化伴心力衰竭的报道很少。在此，我们报告了一例腹膜后纤维化伴大量纵隔受累，病变延伸至胸膜和心包，导致胸膜心包积液及扩张型心肌病的病例。甲基强的松龙治疗后，收缩功能障碍得到改善，心包积液消失。
