Kidney Week

Abstract: SA-PO0749

Dialysis-Requiring AKI Due to Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits Successfully Treated with Clone-Directed Therapy

Session Information

• Glomerular Diseases: Lupus Nephritis, Monoclonal Gammopathy-Related Disease, and Transplantation
  October 24, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Maekawa, Shohei, Kyoto Daigaku, Kyoto, Kyoto Prefecture, Japan

Shohei Maekawa, MD

• Yamamoto, Shinya, Kyoto Daigaku, Kyoto, Kyoto Prefecture, Japan

Shinya Yamamoto, MD, PhD

• Kotani, Mina, Kyoto Daigaku, Kyoto, Kyoto Prefecture, Japan

Mina Kotani, MD

• Suzuki, Haruka, Kyoto Daigaku, Kyoto, Kyoto Prefecture, Japan

Haruka Suzuki, MD

• Yanagita, Motoko, Kyoto Daigaku, Kyoto, Kyoto Prefecture, Japan

Motoko Yanagita, MD, PhD

Introduction

Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a form of monoclonal gammopathy of renal significance. Although PGNMID is considered a clone-driven disease, detectable clones are found in only 20–30% of patients, and plasma cell- or B-cell–targeted therapies have shown efficacy in selected cases. We herein report a case of PGNMID without a detectable clone that relapsed after viral enteritis, presenting with severe AKI.

Case Description

A 60-year-old man presented in year X−2 with lower leg edema. Laboratory findings showed serum creatinine of 1.2 mg/dL, serum albumin of 2.9 g/dL, microscopic hematuria, and a urine protein-to-creatinine ratio of 7.1 g/gCr. Kidney biopsy revealed IgG3λ-type PGNMID. No monoclonal protein was detected in serum or urine. After corticosteroid therapy, partial remission was maintained.
In year X, he developed infectious enteritis complicated by prerenal AKI, which improved with fluid replacement. Two weeks later, however, he developed recurrent edema and relapse of nephrotic syndrome, with serum creatinine of 3.4 mg/dL, serum albumin of 2.4 g/dL, positive urine occult blood, and proteinuria of 5.5 g/gCr. A repeat kidney biopsy showed worsening endocapillary hypercellularity, mesangial proliferation, and narrowing of glomerular capillary lumina, consistent with exacerbation of IgG3λ-type PGNMID. No monoclonal protein or pathogenic clone was detected in serum, urine, or bone marrow.
High dose corticosteroid therapy was ineffective, and kidney function progressively deteriorated, with serum creatinine rising to 10.9 mg/dL, and hemodialysis was started. After initiation of clone-directed therapy with daratumumab and bortezomib, urine output increased within two weeks. Hemodialysis was successfully discontinued, with serum creatinine of 2.5 mg/dL and proteinuria of 2.0 g/gCr. He remained on outpatient clone-directed therapy.

Discussion

Clinicians should recognize that infection-triggered plasma cell or B-cell responses may exacerbate PGNMID and lead to severe AKI, even in the absence of a detectable clone. This case highlights the potential efficacy of clone-directed therapy for recurrent PGNMID without detectable clones, warranting careful follow-up and active therapeutic consideration in severe cases.