{"title":"Monoclonal gammopathy of renal significance (MGRS): retrospective monocentric analysis of clinical outcomes and treatment strategies.","authors":"Pasquale Esposito, Lucia Macciò, Antonia Cagnetta, Francesca Costigliolo, Emilio Venturelli, Elisa Russo, Marco Gallo, Debora Soncini, Francesca Viazzi, Roberto Massimo Lemoli, Michele Cea","doi":"10.1007/s10238-025-01646-7","DOIUrl":null,"url":null,"abstract":"<p><p>Monoclonal Gammopathy of Renal Significance (MGRS) is a group of rare disorders in which monoclonal proteins cause kidney damage. Due to its rarity, ongoing research is vital to refine diagnostics, enhance treatment, and improve outcomes. This retrospective study analyzed 34 patients with renal biopsy-proven MGRS-defining lesions. Patients were divided into two subgroups: kidney-limited AL amyloidosis (MGRS-A, 44%, n = 15) and other MGRS (MGRS-NA, 56%, n = 19). Key outcomes included progression-free survival and overall survival. Baseline characteristics such as histopathology, plasma cell percentage, kidney function, and proteinuria were documented alongside initial treatments, and hematologic and renal response. Distinct differences were observed between the two groups: MGRS-NA was primarily associated with glomerular lesions, while MGRS-A exhibited broader kidney involvement. Treatment varied: bortezomib for plasma cell-driven cases and rituximab for B-cell-related conditions. Anemia was the most common side effect (71%), associated with treatment intensity. Despite similar overall survival outcomes, MGRS-A followed a more aggressive course, with a shorter time from diagnosis to death (206 vs. 728 days). Renal and hematologic responses were comparable between the groups, although baseline factors such as hemoglobin and CRP levels were predictive of mortality. These findings underscore the need for more precise characterization and standardized criteria to optimize the management of MGRS.</p>","PeriodicalId":10337,"journal":{"name":"Clinical and Experimental Medicine","volume":"25 1","pages":"118"},"PeriodicalIF":3.2000,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12000252/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical and Experimental Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s10238-025-01646-7","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"MEDICINE, RESEARCH & EXPERIMENTAL","Score":null,"Total":0}
引用次数: 0
Abstract
Monoclonal Gammopathy of Renal Significance (MGRS) is a group of rare disorders in which monoclonal proteins cause kidney damage. Due to its rarity, ongoing research is vital to refine diagnostics, enhance treatment, and improve outcomes. This retrospective study analyzed 34 patients with renal biopsy-proven MGRS-defining lesions. Patients were divided into two subgroups: kidney-limited AL amyloidosis (MGRS-A, 44%, n = 15) and other MGRS (MGRS-NA, 56%, n = 19). Key outcomes included progression-free survival and overall survival. Baseline characteristics such as histopathology, plasma cell percentage, kidney function, and proteinuria were documented alongside initial treatments, and hematologic and renal response. Distinct differences were observed between the two groups: MGRS-NA was primarily associated with glomerular lesions, while MGRS-A exhibited broader kidney involvement. Treatment varied: bortezomib for plasma cell-driven cases and rituximab for B-cell-related conditions. Anemia was the most common side effect (71%), associated with treatment intensity. Despite similar overall survival outcomes, MGRS-A followed a more aggressive course, with a shorter time from diagnosis to death (206 vs. 728 days). Renal and hematologic responses were comparable between the groups, although baseline factors such as hemoglobin and CRP levels were predictive of mortality. These findings underscore the need for more precise characterization and standardized criteria to optimize the management of MGRS.
单克隆肾性伽玛病(Monoclonal Gammopathy of Renal Significance, MGRS)是一类由单克隆蛋白引起肾损害的罕见疾病。由于其罕见性,正在进行的研究对于改进诊断、加强治疗和改善结果至关重要。本回顾性研究分析了34例肾活检证实的mgrs定义病变。患者分为两个亚组:肾限制性AL淀粉样变(MGRS- a, 44%, n = 15)和其他MGRS (MGRS- na, 56%, n = 19)。主要结局包括无进展生存期和总生存期。基线特征,如组织病理学、浆细胞百分比、肾功能和蛋白尿,与初始治疗、血液学和肾脏反应一起记录。两组之间观察到明显的差异:MGRS-NA主要与肾小球病变相关,而MGRS-A表现出更广泛的肾脏病变。治疗方法多种多样:硼替佐米用于浆细胞驱动病例,利妥昔单抗用于b细胞相关疾病。贫血是最常见的副作用(71%),与治疗强度有关。尽管总体生存结果相似,但MGRS-A的病程更具侵袭性,从诊断到死亡的时间更短(206天对728天)。尽管血红蛋白和CRP水平等基线因素可预测死亡率,但两组之间的肾脏和血液学反应具有可比性。这些发现强调需要更精确的表征和标准化标准来优化MGRS的管理。
期刊介绍:
Clinical and Experimental Medicine (CEM) is a multidisciplinary journal that aims to be a forum of scientific excellence and information exchange in relation to the basic and clinical features of the following fields: hematology, onco-hematology, oncology, virology, immunology, and rheumatology. The journal publishes reviews and editorials, experimental and preclinical studies, translational research, prospectively designed clinical trials, and epidemiological studies. Papers containing new clinical or experimental data that are likely to contribute to changes in clinical practice or the way in which a disease is thought about will be given priority due to their immediate importance. Case reports will be accepted on an exceptional basis only, and their submission is discouraged. The major criteria for publication are clarity, scientific soundness, and advances in knowledge. In compliance with the overwhelmingly prevailing request by the international scientific community, and with respect for eco-compatibility issues, CEM is now published exclusively online.