慢性间质性肺炎伴髓过氧化物酶特异性抗中性粒细胞胞浆自身抗体(MPO-ANCA)的Goodpasture综合征1例。

Nihon Kyobu Shikkan Gakkai zasshi Pub Date : 1997-12-01
A Inoue, N Ino-oka, K Konishi, Y Shindoh, T Suzuki, Y Ono
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引用次数: 0

摘要

一位55岁男性于1996年2月因咯血、贫血进展及肾功能衰竭入院。他被诊断为特发性间质性肺炎,自1988年以来一直在一家地区医院接受随访。入院后第三天,患者突然大量咯血。血清中检测到抗gbm抗体,诊断为Goodpasture综合征。同时检测到高滴度的MPO-ANCA。开始类固醇脉冲治疗、免疫抑制治疗和血浆置换,但由于严重低氧血症和多器官衰竭,他于住院第28天死亡。尸检后组织学检查显示月牙状肾小球肾炎伴肾小球毛细血管壁IgG线状沉积,间质性肺炎伴大量肺泡出血。提示在该患者中,除抗gbm抗体外,循环MPO-ANCA可能参与了good牧草综合征月牙状肾小球肾炎及肺泡出血的进展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[A case of Goodpasture's syndrome with myeloperoxidase specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA) during chronic interstitial pneumonia].

A 55-year-old man was admitted to our hospital with of hemoptysis, progression of anemia and renal failure in February, 1996. Idiopathic interstitial pneumonia had been diagnosed and he had been followed at a regional hospital since 1988. On the third day after admission, he suffered from sudden and massive hemoptysis. Goodpasture's syndrome was diagnosed because anti-GBM antibody was detected in serum. A high titer of MPO-ANCA was also recognized simultaneously. Steroid pulse therapy, immunosuppressive therapy, and plasmapheresis were begun, but he died on the 28th hospital day because of severe hypoxemia and multi-organ failure. Histological examination after autopsy revealed crescentic glomerulonephritis with linear deposition of IgG in the glomerular capillary wall, and interstitial pneumonia accompanied by massive alveolar hemorrhage. It was suggested that in this patient, not only anti-GBM antibody but also circulating MPO-ANCA might have participated in the progression of the crescentic glomerulonephritis and alveolar hemorrhage observed in Goodpasture's syndrome.

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