Kitaura Keisuke, Miyagawa Tomoko, Asano Kenichiro, Oouchi Sachiko, Miki Takashi, Fujisawa Takashi, Ishida Kenjiro
Department of Internal Medicine, Nippon Steel Hirohata Hospital, Hyogo.
Intern Med. 2006;45(20):1177-82. doi: 10.2169/internalmedicine.45.1703. Epub 2006 Nov 15.
The patient was a 42-year-old woman diagnosed as having MCTD and Sjögren's syndrome in 1989, and who was taking oral prednisolone. Proteinuria and microscopic hematuria were pointed out for the first time in December 2004. She was referred to our hospital because of massive hemoptysis. Advanced renal failure, anemia and pulmonary alveolar hemorrhage were diagnosed on admission. She was positive for serum MPO-ANCA. The patient was started on a therapy that included steroids, cyclophosphamide and plasmapheresis. However, her respiratory condition was untreatable and she died on the 16th day of hospitalization. The autopsy revealed alveolar hemorrhage in the lungs and crescentic glomerulonephritis. This patient was considered as a rare case of MCTD associated with MPO-ANCA-positive microscopic polyangiitis.
该患者为一名42岁女性，1989年被诊断患有混合性结缔组织病（MCTD）和干燥综合征，一直在口服泼尼松龙。2004年12月首次发现蛋白尿和镜下血尿。因大量咯血转诊至我院。入院时诊断为晚期肾衰竭、贫血和肺泡出血。血清髓过氧化物酶抗中性粒细胞胞浆抗体（MPO-ANCA）呈阳性。患者开始接受包括类固醇、环磷酰胺和血浆置换在内的治疗。然而，她的呼吸状况无法治疗，于住院第16天死亡。尸检显示肺部有肺泡出血和新月体性肾小球肾炎。该患者被认为是与MPO-ANCA阳性显微镜下多血管炎相关的罕见MCTD病例。
