Omura Yoko, Yoshioka Katsunobu, Tsukamoto Yuka, Maeda Isseki, Morikawa Takashi, Konishi Yoshio, Inoue Takeshi, Sato Toshihiko
Department of Internal Medicine, Osaka City General Hospital, Osaka.
Intern Med. 2006;45(7):461-4. doi: 10.2169/internalmedicine.45.1601. Epub 2006 May 1.
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.
一名70岁男性，此前已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。其病情恶化，遂进行了心包造口术。心包活检显示明显纤维化伴淋巴细胞浸润，这与RPF的表现相同。诊断为多灶性纤维硬化症。尽管进行了积极治疗，但他最终死于心血管衰竭的临床症状。尸检标本显示多个器官有纤维化增生伴淋巴细胞浸润。即使RPF导致的尿路梗阻成功解除，这些患者仍需长期随访，因为可能会出现心包纤维化等其他致命并发症。
