Monoclonal gammapathy of renal significance (MGRS) at the current state: terminology, diagnosis and treatment

Abstract

<jats:p>In this article we discussed the current state of monoclonal gammapathy of renal significance (Monoclonal Gammopathy of Renal Significance MGRS) and revealed problems of B-cell clone secreting nephrotoxic monoclonal immunoglobulin identification. We followed 276 patients with monoclonal gammapathy including patients with non-amyloid nephropathy. The majority of patients had systemic AL-amyloidosis. We established better survival of the treated patients with systemic AL-amyloidosis in comparison with retrospective untreated cohort. We considered current treatment of patients with non-amyloid nephropathy and focused on the crucial role of multidisciplinary approach in management of these patients.</jats:p>

Monoclonal gammopathy of renal significance (MGRS): Real‐world data on outcomes and prognostic factors

“…This is the largest report of biopsy‐proven MGRS 16–25 . The results comprise the real clinical practice of MGRS management, mainly in the past decade.…”

“…The diagnostic criteria was consistent with the consensus statement from the IKMG 12 . All patients included in the analysis required local hematopathological confirmation by renal biopsy for one of the following pathological diagnoses: AL amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), proliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposition disease (PGNMID), monoclonal fibrillary GN, immunotactoid GN, cryoglobulinemic GN, light chain proximal tubulopathy (LCPT), crystal‐storing histiocytosis, C3 glomerulopathy with monoclonal gammopathy, and thrombotic microangiopathy 22–27 . Light‐chain cast nephropathy was excluded since it was considered a myeloma‐defining event 6 .…”

“…MGRS is commonly underdiagnosed due to the rarity and lack of familiarity. The clonal deposition of monoclonal immunoglobulin does not respond to immunosuppression as in other nephropathies, tends to progress or relapse after kidney transplant, leading to end‐stage renal disease 15–22 . For all these reasons, the literature lacks the true incidence and optimal treatment of MGRS.…”