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Abstract: PO1548

Baseline Characteristics of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits in the International Kidney Registry Consortium (K-REG)

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Quinn, Ghazal Z., University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, United States
  • Susanibar-Adaniya, Sandra, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, United States
  • Lau, Stacey, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Kallem, Radhakrishna Reddy, University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Hogan, Jonathan, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, United States

Group or Team Name

  • The International Kidney Registry Consortium (K-REG) and the International Kidney and Monoclonal Gammopathy (IKMG) Research Group
Background

Proliferative glomerulonephritis with monoclonal Ig deposits (PGNMID) is a monoclonal gammopathy of renal significance-associated lesion unique for low rates of paraprotein and clone detection. Prognosis is poor with 30% of patients progressing to ESKD, but recent data suggest improved outcomes with clone-directed therapy regardless of clone-detection status. Here, we present the baseline kidney and hematologic characteristics of patients with PGNMID from the International Kidney Registry Consortium (K-REG).

Methods

PGNMID cases were identified retrospectively by K-REG sites. Baseline demographic, clinical, kidney biopsy, hematologic and treatment data were analyzed and stratified by presence or absence of clone detected, as well conservative vs. non-specific immunosuppression vs. clone-directed therapy prescribed.

Results

134 patients from 15 sites in 3 countries with PGNMID were included, 13% of which had been included in previously published studies. The mean age was 57 (SD 17) years, 49 % of whom were female and 72% Caucasian. The median baseline eGFR was 35 (IQR 21-52) ml/min/1.73m2 and median proteinuria 3.9 (IQR 1.5-7.2) g/day. IgG kappa was the most common involved paraprotein (61%). 30% of patients had a detectable circulating paraprotein by SPEP or sIFE and 11% by UPEP or uIFE. 75% of patients underwent a bone marrow biopsy. Overall, a clone was detected in 19% of patients. There were no significant differences in age, sex, race, baseline eGFR, or proteinuria based on clone or paraprotein-detection status. Clone-directed therapy was prescribed in 55% of patients, non-specific immunosuppression to 15% of patients and conservative therapy in 40% of patients. Clone-directed therapy was prescdribed in 82% of patients with a detectable clone vs. 52% of patients without a detectable clone(p=0.011).

Conclusion

In this large series of PGNMID, a minority of patients had detectable clone. Baseline demographic and clinical characteristics were not different based on clone-detection status but treatment varied significantly based on clone-detection status. Additional analyses of the K-REG cohort will provide insight into renal and hematologic responses.