非典型抗肾小球基底膜（glomerular basement membrane，GBM）肾炎罕见，伴有膜增生病变的更为少见，目前对其治疗方案尚未明确。该文报道1例抗GBM抗体阴性，但肾组织活检提示“肾小球膜增生病变伴IgG线样阳性”的非典型抗GBM肾炎病例。该患者以大量蛋白尿、血尿、水肿、肾功能损伤为主要特征，排除继发因素后经肾组织活检诊断为“非典型抗GBM肾炎”。给予糖皮质激素联合环磷酰胺治疗后，患者出现肺部感染及急性左心衰竭，转院行规律血液透析治疗。后期发展至终末期肾脏病阶段，继续接受规律血液透析治疗。

Atypical anti-glomerular basement membrane (GBM) disease is rare, and the atypical anti-GBM disease with membrane hyperplasia lesion is even rarer. The treatment plan for it is not clear. This article reports a case of atypical anti-GBM disease with a negative anti-GBM antibody test, but renal tissue biopsy showed “glomerular membrane hyperplasia lesions with positive IgG linearity”, which provides a reference for clinical diagnosis and treatment. The patient exhibited massive proteinuria, hematuria, edema, and renal impairment. After ruling out secondary factors, the patient was diagnosed with “atypical anti-GBM nephritis” by renal tissue biopsy, and was treated with glucocorticoids combined with cyclophosphamide, after which the patient developed a lung infection and acute left heart failure, and received regular hemodialysis treatment. Then the patient progressed to the stage of end-stage renal disease and continued to receive regular hemodialysis treatment.