Membranous glomerulonephritis is a manifestation of IgG4-related disease
Membranous glomerulonephritis is a manifestation of IgG4-related disease
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DOI：
10.1038/ki.2012.382
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发表时间：
2013-03-01
影响因子：
19.6
通讯作者：
Cornell, Lynn D.
中科院分区：
文献类型：
--
作者：
Alexander, Mariam P.;Larsen, Christopher P.;Cornell, Lynn D.
IgG4相关疾病（IgG4-RD）是一种全身性免疫介导的疾病，通常表现为几乎可以影响任何器官系统的纤维炎性肿块。IgG4-RD的肾脏受累通常表现为IgG4相关的肾小管间质性肾炎，但也有膜性肾小球肾炎（MGN）的病例。在这里，我们提出了一个系列的9例患者（平均年龄在诊断58岁）与MGN与IgG4-RD。所有患者在活检时均显示MGN，表现为蛋白尿（平均8.3 g/天），大多数患者血清肌酐升高（平均2.2 mg/dl）。7例患者已知IgG4-RD累及肾外，5例患者并发IgG4相关肾小管间质性肾炎。免疫组织化学分析磷脂酶A2受体，标志物的主要MGN，是阴性的所有8个活检检查。7例随访（平均39个月）的患者中有6例接受了免疫抑制剂治疗; 1例未经治疗的患者发生终末期肾病并接受了移植，移植后12年无复发。所有6例接受治疗的患者均显示蛋白尿减少（平均1.2 g/天），大多数患者显示血清肌酐降低（平均1.4 mg/dl）。因此，MGN应包括在IgG4-RD谱中，并应在蛋白尿IgG4-RD患者中进行怀疑。相反，MGN患者和适当的临床病史应评估IgG4-RD。Kidney International（2013）83，455 - 462; doi：10.1038/ki.2012.382; 2012年12月19日在线发表
IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease that typically manifests as fibro-inflammatory masses that can affect nearly any organ system. Renal involvement by IgG4-RD usually takes the form of IgG4-related tubulointerstitial nephritis, but cases of membranous glomerulonephritis (MGN) have also been described. Here we present a series of 9 patients (mean age at diagnosis 58 years) with MGN associated with IgG4-RD. All patients showed MGN on biopsy, presented with proteinuria (mean 8.3 g/day), and most had elevated serum creatinine (mean 2.2 mg/dl). Seven patients had known extrarenal involvement by IgG4-RD, with 5 patients having concurrent IgG4-related tubulointerstitial nephritis. Immunohistochemical analysis for the phospholipase A2 receptor, a marker of primary MGN, was negative in all 8 biopsies so examined. Six of 7 patients with available follow-up (mean 39 months) were treated with immunosuppressive agents; one untreated patient developed end-stage renal disease and underwent transplantation, without recurrence at 12 years after transplant. All 6 treated patients showed decreased proteinuria (mean 1.2 g/day), and most showed decreased serum creatinine (mean 1.4 mg/dl). Thus, MGN should be included in the spectrum of IgG4-RD and should be suspected in proteinuric IgG4-RD patients. Conversely, patients with MGN and an appropriate clinical history should be evaluated for IgG4-RD. Kidney International (2013) 83, 455-462; doi:10.1038/ki.2012.382; published online 19 December 2012