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Abstract: FR-PO902

Proliferative Glomerulonephritis with Non-Organised Monoclonal Immunoglobulin Deposits (PGNMID): A Single-Centre Retrospective Study

Session Information

Category: Glomerular Diseases

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Blakey, Sarah, Imperial College Healthcare NHS Trust, London, United Kingdom
  • Duncan, Neill D., Hammersmith Hospital, London, United Kingdom
  • Tam, Frederick W.K., Imperial College Kidney and Transplant Institute, London, United Kingdom
  • Cairns, Tom, Imperial College Healthcare NHS Trust, London, United Kingdom
  • Levy, Jeremy B., Imperial College Healthcare NHS Trust, London, United Kingdom
  • Cook, H. Terence, Imperial College London, London, United Kingdom
  • Chaidos, Aristeidis, Imperial College London, London, United Kingdom
  • Roufosse, Candice A., Imperial College London, London, United Kingdom
  • Kousios, Andreas, Imperial College Healthcare NHS Trust, London, United Kingdom
Background

PGNMID is a rare glomerular disease, most often seen in the context of MGRS. At present, optimal treatment is not established.

Methods

All native renal biopsies performed at the Hammersmith Hospital between 2006 and 2017 were analysed. 15 cases of PGNMID were identified. Baseline characteristics and clinical outcomes during follow-up to January 2019 are summarised in the Table.

Results

Mean age was 61, 31% were men, mean eGFR was 49 mL/min/1.73m2 and mean uPCR 384 mmol/mol. A circulating paraprotein was detectable in 5 (33%) of 15 patients. Most (73%) underwent bone marrow aspiration and trephine (BMAT), with a clone identified in two of 11. One had a plasma cell clone and was not immunosuppressed, having presented at end stage with an eGFR of 7mL/min/1.73m2 and 50% IFTA. The second patient had a B cell clone, with an eGFR of 83 mL/min/1.73m2 and 0% IFTA, and achieved remission of proteinuria and stabilisation of eGFR with prednisolone and rituximab.

Three (20%) patients had a detectable paraprotein but no clone on BMAT, and all received treatment. Two progressed to ESRD despite steroids, rituximab and cyclophosphamide. One patient initially responded to steroids, MMF, rituximab and bortezomib, but relapsed following cessation of bortezomib due to peripheral neuropathy.

10 of 15 (67%) patients had no detectable paraprotein at diagnosis. Of these, 89% had partial or complete renal remission. Treatment of this group was variable; none had clone-directed therapy. One case was lost to follow-up post renal biopsy.

Conclusion

Our cohort of PGNMID patients corroborates the previously-described low rate of detection of circulating paraprotein and pathogenic clones on BMAT. Further collaborative studies are required to establish the safety and efficacy of clone-directed therapy, and to guide optimal management in patients with PGNMID.