Polyclonal Immunotactoid Glomerulopathy Associated with Monoclonal Gammopathy of IgM Type and Underlying Plasmacellular Disease: Successful Treatment with Rituximab Alone

IF 0.7 Q4 UROLOGY & NEPHROLOGY Case Reports in Nephrology and Dialysis Pub Date : 2022-04-25 DOI:10.1159/000524131
Wolfgang Neukirchen, A. Oesterling, D. Wennmann, B. Heitplatz, Peter Ritter, H. Merz, Veit Busch
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Abstract

Immunotactoid glomerulopathy (ITG) occurs infrequently and is characterized by organized IgG containing deposits. It most usually manifests as a concomitant disease of a broad spectrum of oncologic entities. We here present an exceptional case of ITG without glomerular light chain restriction secondary to a IgM kappa type monoclonal gammopathy of undetermined significance. Due to nephrotic syndrome and deterioration of kidney function a rituximab monotherapy was initiated without targeting the plasmacellular augmentation, which was confirmed as the underlying process. The treatment led to a long-term improvement of proteinuria and stabilization of glomerular filtration rate. Its therapeutic effect has to be attributed to immunomodulatory capacities and targeting of podocytes rather than to be interpreted as directed against a bone marrow or glomerular clone. We conclude that rituximab therapy may be a valuable part of the therapeutic options in ITG irrespective of the underlying oncologic entity.
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IgM型单克隆免疫球蛋白病相关的多克隆免疫球蛋白病变和潜在的脱细胞性疾病:单独使用利妥昔单抗的成功治疗
免疫因子样肾小球病(ITG)很少发生,其特征是有组织的含有IgG的沉积。它通常表现为广泛的肿瘤实体的伴随性疾病。我们在此报告一例罕见的ITG,没有继发于IgM kappa型单克隆γ病的肾小球轻链限制,其意义尚不确定。由于肾病综合征和肾功能恶化,开始了利妥昔单抗单药治疗,不针对浆细胞增强,这被证实是潜在的过程。治疗导致蛋白尿的长期改善和肾小球滤过率的稳定。其治疗效果必须归因于免疫调节能力和足细胞的靶向性,而不是被解释为直接针对骨髓或肾小球克隆。我们的结论是,无论潜在的肿瘤实体如何,利妥昔单抗治疗可能是ITG治疗选择的一个有价值的部分。
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来源期刊
CiteScore
1.20
自引率
0.00%
发文量
36
审稿时长
10 weeks
期刊介绍: This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of nephrology and dialysis, including genetic susceptibility, clinical presentation, diagnosis, treatment or prevention, toxicities of therapy, critical care, supportive care, quality-of-life and survival issues. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed.
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