Abstract: PO1498
Spontaneously Resolved Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits
Session Information
- Glomerular Diseases: The Excitement of Clinical Cases
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Shaikhouni, Salma, University of Michigan, Ann Arbor, Michigan, United States
- Peirce, Alexandra Marie, University of Michigan, Ann Arbor, Michigan, United States
- Oliverio, Andrea L., University of Michigan, Ann Arbor, Michigan, United States
- Mariani, Laura H., University of Michigan, Ann Arbor, Michigan, United States
Introduction
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is thought to be a progressive disease, with early studies showing 38% of patients with persistent renal dysfunction and 22% progressing to ESKD within a 30 month follow up period. We present two cases of PGNMID who achieved spontaneous remission without directed therapy.
Case Description
Case 1: A 53 yo woman with hypertension and diabetes presented for evaluation of proteinuria with UPCR 3.86 g/g and normal creatinine. She was asymptomatic, but had an upper respiratory infection 1 month prior. Serologic evaluation for nephrotic range proteinuria was unrevealing. Renal biopsy showed PGNMID with IgG λ light chain deposits. Serum paraprotein was not detected. Bone marrow biopsy showed no concern for malignancy. UPCR decreased to 0.63 within 4 months with no treatment but an ACEi.
Case 2: A 53 yo man with primary biliary cholangitis presented with edema and new hypertension. He was found to have AKI with SCr 1.83 mg/dL,UPCR of 12.9 g/g, and microscopic hematuria. While serum autoimmune markers were notable for mildly elevated anti-dsDNA (75 IU/mL), normal C3 and low C4 (8 mg/dL), renal biopsy revealed PGNMID with IgG λ light chain deposits. A low titer IgM κ was detected in the serum. The patient had diffuse lymphadenopathy on imaging, and two excisional biopsies showed reactive follicular hyperplasia. Bone marrow biopsy was normal. UPCR decreased to 1.23 g/g within 2.5 months of presentation and SCr down to 1.22 without any therapy.
Discussion
These unique cases demonstrate that PGNMID is likely a heterogeneous disease whose pathogenesis and natural history is poorly understood. We hypothesize that an infectious antigen or autoantigen may induce clonal proliferation of B-cells and the formation of transient monoclonal antibodies leading to complement activation and proliferative glomerulonephritis. When the stimulus is controlled, the associated immune response is regulated. Our patients did not require any immunomodulatory therapy, contrary to most recent case series. Recognition of the potential for spontaneous remission in PGNMID has important implications on treatment paradigms for this emerging diagnosis.