Ishiguro Takashi, Takayanagi Noboru, Yamaguchi Shozaburo, Shimizu Yoshihiko, Yanagisawa Tsutomu, Sugita Yutaka, Kawabata Yoshinori
Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Japan.
Intern Med. 2012;51(8):905-9. doi: 10.2169/internalmedicine.51.6518. Epub 2012 Apr 15.
A 73-year-old woman presented to our hospital with scleritis, cough, and hemoptysis. Chest computed tomography showed a mass lesion, multiple ground-glass opacities, and nodules. Bronchoscopy showed a skip lesion of mucosal erosion with white necrotic substance; however, a biopsy specimen of the erosion yielded no characteristic findings. Transbronchial lung biopsy showed alveolar hemorrhage and capillaritis. Proteinase-3 antineutrophil cytoplasmic antibody was elevated to 62.5 U/mL, and she was diagnosed as having Wegener's granulomatosis. Steroid therapy combined with cyclophosphamide was started; however, hemoptysis was not resolved until plasmapheresis therapy was added. Her general condition initially improved, but respiratory insufficiency worsened with fever on the 30th hospital day. The patient subsequently died on the 41st hospital day, and cytomegalovirus infection was suggested as a possible cause of death.
一名73岁女性因巩膜炎、咳嗽和咯血入住我院。胸部计算机断层扫描显示有一个块状病变、多个磨玻璃样混浊及结节。支气管镜检查显示有一处跳跃性病变，表现为黏膜糜烂并伴有白色坏死物质；然而，对糜烂处的活检标本未发现特征性表现。经支气管肺活检显示肺泡出血和毛细血管炎。蛋白酶3抗中性粒细胞胞浆抗体升高至62.5 U/mL，她被诊断为韦格纳肉芽肿。开始给予类固醇治疗联合环磷酰胺；然而，直到加用血浆置换疗法，咯血才得以缓解。她的一般状况最初有所改善，但在住院第30天时，呼吸功能不全因发热而加重。该患者随后于住院第41天死亡，巨细胞病毒感染被认为可能是死亡原因。
