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Abstract: FR-PO614

Rapidly Progressive Glomerulonephritis Associated With Recurrent Cryoglobulinemia in a Patient With Monoclonal Gammopathy of Undetermined Significance

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Neinast, Michael B., Southern Illinois University School of Medicine, Springfield, Illinois, United States
  • Chen, Xueguang (Gary), Southern Illinois University School of Medicine, Springfield, Illinois, United States
Introduction

Mixed cryoglobulinemia is most often associated with HCV infection, and less commonly, monoclonal gammopathies. Rapidly progressive glomerulonephritis is a common pathway of disease in these patients and early diagnosis is imperative for renal recovery. We present the case of recurrent cryoglobulinemia in a patient with MGUS resulting in RPGN after rituximab suppression therapy.

Case Description

A 50-year-old male presented for evaluation of progressive abdominal pain and anorexia. He has a history of cryoglobulinemia and GN three years prior with renal biopsy showing tubular and interstitial inflammation with immune-complex deposition not completely consistent with either monoclonal gammopathy or cryoglobulinemia. It was treated successfully with three courses of rituximab with resultant CKD3b. Further workup revealed MGUS on SPEP and bone marrow biopsy. A repeat bone marrow biopsy two months prior showed normocellular bone marrow with minute population of kappa monocytic plasma cells consistent with his known MGUS. On current admission, his creatinine was 10.2 g/dL with severe proteinuria of 8,216g/24 hours. Other lab values were significant for persistent IgM kappa monoclonal gammopathy on SPEP, low C3 and C4, significantly elevated RF with negative anti-CCP, and cryoglobulin level of 6%, consistent with mixed type II cryoglobulinemia. Renal biopsy showed cryoglobulinemic glomerulonephritis with focal crescentic formation involving 25% of glomeruli, severe tubulointerstitial nephritis, moderate interstitial fibrosis, and tubular atrophy. The patient was started on nine total plasmapheresis sessions over three weeks and dialysis concurrently, with rituximab infusion weekly for four weeks. Repeat RF decreased from 922 to 142 and cryoglobulin level was negative after treatment was completed. Ultimate prognosis is yet to be determined. He is continued on dialysis at this time.

Discussion

MGUS is a well-established part of the monoclonal gammopathy spectrum. MGRS was a recently proposed portion of this spectrum in 2012 to underline the need for more aggressive treatment in this population. While this case re-demonstrates promise of rituximab treatment for initial cryoglobulinemia associated with MGUS, it further underlines the need for improved diagnosis of the renal significance in this spectrum to prevent further kidney damage and possible ESRD.