Department of Medicine, Charles University 2nd School of Medicine and University Hospital Motol, Prague, Czech Republic.
Ren Fail. 2012;34(2):244-6. doi: 10.3109/0886022X.2011.647208. Epub 2012 Jan 20.
A 47-year-old man was admitted to hospital for migratory joint pain, fatigue, and cough with bloody sputum and proteinuria with increased serum creatinine level. Diagnosis of Wegener's granulomatosis was established. During follow-up, the vena cava superior syndrome developed. The patient died of respiratory failure after 12 years of follow-up. The autopsy revealed rigid, whitish, 12 mm thick tissue, which embedded and compressed the large vessels upwards from their origin in the heart, thus causing vena cava superior syndrome. This tissue was composed of fibrous material without inflammatory cellulization. We consider this fibrous tissue as a manifestation of fibrosing mediastinitis that may or may not share pathogenesis with Wegener's granulomatosis.
一位 47 岁男性因游走性关节痛、乏力、咳嗽、咯血和蛋白尿伴血清肌酐水平升高而入院。诊断为韦格纳肉芽肿病。随访期间发生上腔静脉综合征。患者在随访 12 年后死于呼吸衰竭。尸检显示，有 12 毫米厚的坚硬、发白的组织，从心脏起源处向上嵌入并压迫大血管，导致上腔静脉综合征。该组织由纤维性物质组成，无炎症性细胞浸润。我们认为这种纤维组织是纤维性纵隔炎的表现，其发病机制可能与韦格纳肉芽肿病有关，也可能无关。
