Department of Nephrology and Hypertension, University Medical Center Utrecht, F03.2.22, P.O. Box 85500, 3508 GA, Utrecht, The Netherlands.
Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands.
J Nephrol. 2020 Jun;33(3):611-617. doi: 10.1007/s40620-020-00723-2. Epub 2020 Mar 27.
Monoclonal gammopathy of renal significance (MGRS) encompasses a group of disorders in which a monoclonal immunoglobulin (M-protein) secreted by a B-cell or plasma cell clone causes renal disease. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a form of MGRS where M-protein is deposited in the glomerulus. Although evidence is limited, the current consensus is that therapy for PGNMID should be directed against the underlying clone. However, it is conceivable that there is heterogeneity in the renal prognosis of PGNMID and that not all patients have need for clone-directed therapy. Here, we report two cases of PGNMID with IgM-kappa gammopathy. In one case of a 53-year-old woman the glomerulonephritis resolved without clone-directed therapy. In the other case of a 34-year-old woman clone-directed therapy was discontinued due to adverse effects. Although no hematological response was achieved, the PGNMID resolved. In both cases there are no signs of a recurrent glomerulonephritis in over 3 years of follow-up. Here, we review the literature and suggest that some PGNMID patients have a favorable renal prognosis in whom clone-directed therapy can be withheld or postponed. Further research is warranted to yield predictors to identify these patients and to better understand the disease course of PGNMID.
肾相关单克隆免疫球蛋白血症(MGRS)涵盖了一组疾病,其中单克隆免疫球蛋白(M 蛋白)由 B 细胞或浆细胞克隆分泌,导致肾脏疾病。伴有单克隆免疫球蛋白沉积的增殖性肾小球肾炎(PGNMID)是 MGRS 的一种形式,其中 M 蛋白沉积在肾小球中。尽管证据有限,但目前的共识是,PGNMID 的治疗应该针对潜在的克隆。然而,可以想象,PGNMID 的肾脏预后存在异质性,并非所有患者都需要针对克隆的治疗。在这里,我们报告了两例伴有 IgM-κ 血症的 PGNMID。在一例 53 岁女性中,肾小球肾炎未经克隆靶向治疗而缓解。在另一例 34 岁女性中,由于不良反应而停止了克隆靶向治疗。尽管未达到血液学反应,但 PGNMID 得到缓解。在这两种情况下,随访超过 3 年均未出现复发性肾小球肾炎的迹象。在这里,我们回顾了文献,并提出了一些 PGNMID 患者具有良好的肾脏预后,可以不进行或推迟克隆靶向治疗。需要进一步的研究来预测这些患者,并更好地了解 PGNMID 的疾病过程。