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Abstract: PO1491

Proliferative Glomerulonephritis with Monoclonal IgM Deposits in ANCA Vasculitis

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Kethineni, Rama, University of Utah Health, Salt Lake City, Utah, United States
  • Barry, Marc, University of Utah Health, Salt Lake City, Utah, United States
  • Revelo Penafiel, Monica Patricia, University of Utah Health, Salt Lake City, Utah, United States
  • Kottey, Janame J., University of Utah Health, Salt Lake City, Utah, United States
  • Yadav, Niraj K., University of Utah Health, Salt Lake City, Utah, United States
  • Abraham, Josephine, University of Utah Health, Salt Lake City, Utah, United States
Introduction

Proliferative glomerulonephritis with monoclonal immunoglobin deposition (PGNMID) is a rare entity of unclear etiology that can be occasionally associated with an underlying hematological malignancy. We report a case of PGNMID in a patient with ANCA vasculitis.

Case Description

A 78 yr old female with hypertension presented with a history of recurrent sinusitis over 6 months that was treated with steroids and antibiotics. She had progressive weight loss, fatigue, cough, and dyspnea. Chest X-ray was normal prompting a CT chest, which showed bilateral pulmonary nodules. Her creatinine was 0.96mg/dL. Urine analysis was notable for hematuria. Antineutrophil cytoplasmic antibodies were positive at 1: 640 with myeloperoxidase Abs IgG of 199. Serum protein electrophoresis showed a normal pattern with no monoclonal spike on immunofixation electrophoresis. Her Kappa/Lambda light chain ratio was normal at 1.04, infections were ruled out, and cryoglobulin was not detected. She was started on prednisone 60 mg and had a renal biopsy.

Renal biopsy showed focal segmental necrotizing glomerular lesions, mesangial hypercellularity with deposition of IgM with lambda light chains restriction on immunofluorescence and occasional mesangial and subendothelial granular electron densities on electron microscopy.

She was referred to hematology for concern with monoclonal gammopathy of renal significance and had a negative evaluation on serological tests, bone marrow biopsy and PET CT scan. She was treated with Rituximab. Her creatinine had remained stable with resolution of hematuria and respiratory symptoms.

Discussion

PGNMID is an immune complex glomerulonephritis that is occasionally associated with a hematological malignancy. The pathophysiology remains elusive and treatment can be challenging. We present a case of PGNMID with monoclonal IgM with light lambda chain restriction with an unusual association with ANCA vasculitis.