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抗中性粒细胞胞浆抗体和抗肾小球基底膜抗体相关性血管炎所致单侧弥漫性肺受累

[Unilateral diffuse pulmonary involvement in vasculitis with anti-neutrophil cytoplasmic antibodies and anti-glomerular basement membrane antibodies].

作者信息

Dobos G J, Grotz W, Gondolf K B, Rohrbach R, Peter H H, Schollmeyer P

机构信息

Abteilung Innere Medizin IV, Medizinische Klinik Universität, Freiburg.

出版信息

Dtsch Med Wochenschr. 1994 Aug 5;119(31-32):1071-5. doi: 10.1055/s-2008-1058804.

Abstract

A previously healthy 51-year-old man, hospitalized because of fatigue and joint complaints, was found to have left-sided diffuse pulmonary infiltration and a rapid rise in creatinine concentration. Anti-neutrophil cytoplasmic antibodies (cANCA) and anti-glomerular basement antibodies (anti-GBM) were found in serum. Renal biopsy revealed extracapillary glomerulonephritis with diffuse half-moon formations. Immunohistology showed a diffuse granular pattern for immunoglobulin G, M and complement C3, without linear deposits. Immunosuppressive treatment combined with plasmapheresis brought about rapid clinical improvement and normalization of the lung involvement and renal function, as well as a fall in the cANCA titre. During a follow-up period of 5 years there has been no recurrence of symptoms, although the anti-GBM titre remained strongly positive and the cANCA titre twice rose to abnormal levels.

摘要

一名既往健康的51岁男性,因疲劳和关节不适入院,检查发现左侧肺部弥漫性浸润,肌酐浓度迅速升高。血清中检测到抗中性粒细胞胞浆抗体(cANCA)和抗肾小球基底膜抗体(抗GBM)。肾活检显示为伴有弥漫性半月形形成的毛细血管外肾小球肾炎。免疫组织学显示免疫球蛋白G、M和补体C3呈弥漫性颗粒状模式,无线性沉积。免疫抑制治疗联合血浆置换使临床症状迅速改善,肺部病变和肾功能恢复正常,cANCA滴度下降。在5年的随访期内,尽管抗GBM滴度仍呈强阳性,cANCA滴度两次升至异常水平,但症状未复发。

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