Kalluri R, Meyers K, Mogyorosi A, Madaio M P, Neilson E G
Penn Center for Molecular Studies of Kidney Diseases, University of Pennsylvania, Philadelphia, USA.
J Am Soc Nephrol. 1997 Nov;8(11):1795-800. doi: 10.1681/ASN.V8111795.
A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the alpha 3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.
一名68岁的白种女性因结节性肺部浸润和急性肾衰竭入院。最初考虑为韦格纳肉芽肿病，因为血清中抗中性粒细胞胞浆抗体（c-ANCA）水平升高。肾活检后通过互联网会诊显示为新月形坏死性肾小球肾炎以及免疫球蛋白G（IgG）和补体C3的线性沉积，这是抗肾小球基底膜（GBM）病的典型表现。开始进行血液透析；然而，患者突然发生大量脑出血，在全面治疗生效前死亡。对患者血清的尸检分析显示，针对IV型胶原α3 NC1结构域的IgG滴度很高。在快速进展性肾小球肾炎的情况下，p-ANCA和抗GBM抗体的血清学证据越来越多地被认识到。本文报道的患者具有c-ANCA抗体与抗GBM病的不寻常组合，这种关联引发了关于此类肾损伤发病机制的复杂问题。
