Isobe Shinsuke, Tomosugi Toshihide, Futamura Kenta, Okada Manabu, Hiramitsu Takahisa, Tsujita Makoto, Narumi Shunji, Goto Norihiko, Takeda Asami, Watarai Yoshihiko
Department of Kidney Disease Center, Nagoya Daini Red Cross Hospital, Nagoya, Japan,
Department of Kidney Disease Center, Nagoya Daini Red Cross Hospital, Nagoya, Japan.
Nephron. 2020;144 Suppl 1:49-53. doi: 10.1159/000511625. Epub 2020 Nov 25.
Atypical anti-glomerular basement membrane (GBM) nephritis is a rare variant of the classical anti-GBM antibody disease. Patients present with an undetectable anti-GBM antibody but show linear glomerular basement membrane staining for immunoglobulin. We present a 69-year-old man who underwent a living-donor kidney transplant. The aetiology of the renal failure was a focal segmental glomerulonephritis-like lesion resistant to immunosuppressive therapy. A renal graft biopsy revealed diffuse endocapillary hypercellularity, and mild mesangiolysis with linear GBM staining for IgG. The patient was diagnosed with atypical anti-GBM nephritis since the patient tested negative for circulating anti-GBM antibodies. Treatment involved intravenous methylprednisolone, plasma exchange, and rituximab administration. Protocol graft biopsy performed 1 year after the renal transplant showed a focal segmental glomerulonephritis-like lesion possibly progressing from endocapillary hypercellularity and mesangiolysis. These findings were similar to his native kidney biopsy findings. Although classical recurrent anti-GBM nephritis is rare when a renal transplant is performed after decreased disease activity, this case was considered as a case of recurrent atypical anti-GBM nephritis after renal transplant.
非典型抗肾小球基底膜(GBM)肾炎是经典抗GBM抗体疾病的一种罕见变体。患者抗GBM抗体检测不到,但免疫球蛋白显示肾小球基底膜呈线性染色。我们报告一名69岁接受活体供肾移植的男性。肾衰竭的病因是对免疫抑制治疗耐药的局灶节段性肾小球肾炎样病变。肾移植活检显示弥漫性毛细血管内细胞增多,伴有轻度系膜溶解,IgG呈肾小球基底膜线性染色。由于患者循环抗GBM抗体检测呈阴性,故诊断为非典型抗GBM肾炎。治疗包括静脉注射甲泼尼龙、血浆置换和使用利妥昔单抗。肾移植1年后进行的方案移植肾活检显示局灶节段性肾小球肾炎样病变,可能由毛细血管内细胞增多和系膜溶解发展而来。这些发现与他的自体肾活检结果相似。尽管在疾病活动度降低后进行肾移植时,经典的复发性抗GBM肾炎很少见,但该病例被认为是肾移植后复发性非典型抗GBM肾炎的病例。