Multifocal fibrosclerosis combined with idiopathic retro-peritoneal and pericardial fibrosis.
Multifocal fibrosclerosis combined with idiopathic retro-peritoneal and pericardial fibrosis.
复制标题
多灶性纤维硬化症合并特发性腹膜后纤维化和心包纤维化。
DOI：
10.2169/internalmedicine.45.1601
复制
发表时间：
2006
影响因子：
1.2
通讯作者：
Toshihiko Sato
中科院分区：
文献类型：
--
作者：
Y. Omura;K. Yoshioka;Y. Tsukamoto;Isseki Maeda;T. Morikawa;Y. Konishi;Takeshi Inoue;Toshihiko Sato
一位70岁的男性，被诊断为腹膜后纤维化（RPF），因呼吸困难入院。影像学检查显示大量心包积液。他的病情恶化，并进行心包造口术。心包活检显示明显的纤维化伴淋巴细胞浸润，与RPF结果相同。诊断为多灶性纤维硬化症。尽管进行了积极的治疗，他还是死于心血管衰竭的临床体征。尸检标本显示多个器官纤维化增生伴淋巴细胞浸润。即使在成功地对RPF的尿路梗阻进行减压后，由于可能发生其他致命并发症（如心包纤维化），这些患者仍需要长期随访。
A 70-year-old man who had been diagnosed with retroperitoneal fibrosis (RPF) was admitted to our hospital complaining of dyspnea. Imaging studies showed massive pericardial effusion. His condition deteriorated and pericardiostomy was performed. A biopsy of the pericardium revealed marked fibrosis with infiltration of lymphocytes, which was identical to RPF findings. A diagnosis of multifocal fibrosclerosis was made. Despite aggressive treatment, he died with clinical signs of cardiovascular failure. The autopsy specimen revealed proliferation of fibrosis with infiltration of lymphocytes in multiple organs. Even after successful decompression of urinary obstruction for RPF, long-term follow-up is necessary in these patients because of the possibility of other fatal complications such as pericardial fibrosis.