Li Manna, Xu Gaosi
Department of Nephrology, the Second Affiliated Hospital of Nanchang University, Jiangxi, China.
Clin Kidney J. 2021 Dec 14;15(6):1041-1048. doi: 10.1093/ckj/sfab269. eCollection 2022 Jun.
As aging increases, monoclonal gammopathy is becoming more common and monoclonal gammopathy of renal significance (MGRS) is gaining attention due to frequent renal involvement. Within MGRS, proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a special category. The disease was first described in 2004 and the research history on it is relatively short. Compared with other MGRS, the detection rate of circulating clones is lower in patients with PGNMID, which is easy to miss and misdiagnose in clinical work. In this review, the etiology and clinical features of PGNMID are discussed. It is noted that PGNMID is associated not only with MGRS, but also with malignancy, infection and other factors. PGNMID is not a disease exclusive to the elderly-young people can also develop this disease. Due to the low detection rate of circulating clones in most patients, confirmation of the disease needs to be combined with renal pathology, which emphasizes the importance of completing light and heavy chain subtype staining. Treatment options for patients with PGNMID differ by etiology. For MGRS-associated PGNMID, the current treatment is primarily empirical and more research evidence is needed to fill the treatment gap.
随着年龄增长，单克隆丙种球蛋白病越来越常见，而具有肾脏意义的单克隆丙种球蛋白病（MGRS）因常累及肾脏而受到关注。在MGRS中，伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎（PGNMID）是一个特殊类型。该疾病于2004年首次被描述，其研究历史相对较短。与其他MGRS相比，PGNMID患者循环克隆的检出率较低，在临床工作中容易漏诊和误诊。在这篇综述中，讨论了PGNMID的病因和临床特征。值得注意的是，PGNMID不仅与MGRS有关，还与恶性肿瘤、感染等因素有关。PGNMID并非老年人特有的疾病——年轻人也可能患此病。由于大多数患者循环克隆的检出率较低，该病的确诊需要结合肾脏病理检查，这凸显了完成轻链和重链亚型染色的重要性。PGNMID患者的治疗方案因病因不同而异。对于与MGRS相关的PGNMID，目前的治疗主要是经验性的，需要更多的研究证据来填补治疗空白。
