Management of monoclonal gammopathy of renal significance: treatment standard

Abstract Monoclonal gammopathy of renal significance (MGRS) is a heterogeneous group of clonal B-cell or plasma cell proliferative disorders that cause kidney injury without meeting criteria for overt malignancy. Advances in diagnostic techniques have enhanced the identification and classification of MGRS lesions. Clone-directed therapies, such as proteasome inhibitors, anti-CD38, and anti-CD20 monoclonal antibodies, are now central to treatment, targeting a deep hematologic response that is strongly associated with improved renal outcomes and reduced risk of recurrence following kidney transplantation. Despite these advances, management remains challenging in patients without detectable clones. This review highlights recent progress in the diagnosis and treatment of MGRS, with an emphasis on therapeutic strategies, transplant considerations, and evolving clinical challenges.