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.