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Abstract: FR-PO1084

Chemotherapy Response and 2 Years Follow-Up of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits (PGNMID)

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Valjalo, Ricardo, Hospital del Salvador, Santiago, Chile
  • Pena, Camila, Hospital del Salvador, Santiago, Chile
  • Mendez, Gonzalo P., Pontificia Universidad Catolica de Chile., Vitacura, SANTIAGO, Chile
Background

The term monoclonal gammopathy of renal significance (MGRS) represents all monoclonal gammopathies that are associated with the development of a kidney disease but do not meet the definition of hematological malignancies. PGNMID is an uncommon MGRS–related glomerular disorder characterized by monoclonal Ig deposits resulting in an endocapillary proliferative or membranoproliferative histological pattern of injury. It usually manifests with proteinuria, microscopic hematuria and renal impairment. Treatment for PGNMID has included RAS blockage, rituximab, chemotherapy and immunosuppressive therapy with variable results. Nonetheless, reports with treatment outcomes are scarce, heterogeneous and retrospective, so the benefits of specific treatment remain unclear. Here we report for first time, the prospective results of 3 cases of PGNMID treated with a cyclophosphamide, thalidomide and dexamethasone (CTD regimen) based oral chemotherapy.

Methods

Prospective analysis. Three cases of PGNMID were identified during 2015 at Hospital del Salvador (Santiago, Chile). The biopsies were analyzed using standard techniques for light microscopy, IF, and EM. The presence of plasma cell dyscrasia and other conditions were evaluated. All patients received CTD regimen and RAS blockage.

Results

Two patients made their debut with impure nephrotic syndrome and rapidly progressive renal failure. Patient 2 had subnephrotic proteinuria and hematuria. In all cases M-protein could not be detected by serum or urine PEP/IF and only κ/λ relation was elevated. All patients had no bone marrow involvement, and autoimmunity study was negative. The IF revealed mesangial deposits restricted to IgG3-kappa in patients 1 and 3, and IgA kappa in patient 2. After CTD regimen, two patients achieved complete remission with no adverse effects and no relapses after 2 years of follow-up. The third patient had a serious pneumonia with severe AKI and dialysis requirement after 8 months of initiating chemotherapy. Thereafter had a serious Clostridium difficile infection, chemotherapy was stopped and the patient continued on dialysis.

Conclusion

The CTD chemotherapy regimen was successful in the treatment of PGNMID attaining long-term complete remission. CTD regimen seems to be an effective treatment, but we must put attention on chemotherapy related adverse effects