Abstract: SA-PO0775
Pediatric Proliferative Glomerulonephritis with Monoclonal IgG Deposits: A Single-Center Case Series Highlighting Phenotypic and Treatment Heterogeneity
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
- Sawaya, Louise Barbara, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
- Varnell, Charles D., Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, United States
Introduction
Pediatric Proliferative Glomerulonephritis with Monoclonal IgG Deposits (PGNMID) is a rare. In adults, daratumumab (anti-CD38) has shown efficacy in PGNMID with and without a detectable clone. There are no treatment guidelines for pediatric PGNMID. This is a single-center retrospective review of pediatric patients with biopsy-confirmed PGNMID. Demographics, biopsy findings, treatment, and labs were extracted.
Case Description
1: 17 year-old female with nephrotic syndrome, initially with immune-complex membranoproliferative GN, with IgG and C3 deposition. She had a nonrevealing bone marrow biopsy and no circulating clone. She was treated with corticosteroids (Cr 0.79, albumin 3.0, and UPC 6.28). She received cyclophosphamide for RPGN with crescentic lesion, daratumumab, and obinutuzumab, and losartan. 22 months after presentation, she had Cr 0.62 and a UPC 1.9.
2: 10 year-old female with Klippel-Trenaunay syndrome and nephrotic syndrome, with undetectably low C3 (<3), C4 10, Cr 0.32, albumin 2.3, and UPC 4.9. Kidney biopsy showed membranoproliferative pattern of GN with IgG3-Lambda and C3 deposition. Bone marrow biopsy was nonrevealing. Treatment has consisted of corticosteroids, MMF, and lisinopril. 5 months after presentation, she had a UPC 0.91, Cr of 0.4, and after 19 months had a UPC 0.6.
3: 12 year-old male with adenylosuccinate lyase deficiency, initially treated with corticosteroids and MMF. Previous biopsy showed proliferative GN with significant immune deposits, and he had a nonrevealing bone marrow biopsy. Repeat biopsy for AKI revealed a membranoproliferative GN with IgG deposition, lambda restricted pattern, and AIN. He remained on corticosteroids, however MMF was discontinued due to recurrent infections. He has progressed to CKD 3a. 40 months after presentation, he had Cr 1.26, albumin 1.3, and UPC 8.67.
Discussion
Among pediatric patients, there is variety in phenotype and treatment. Outcomes ranged from near-remission (2) to CKD 3a progression (3). All 3 patients had negative bone marrow biopsy and no detectable serum clone, in contrast to adult disease. All received steroids and RAAS inhibition if able, but other immunosuppressants were chosen based on individua characteristics. Daratumumab was successfully used in a clone-negative pediatric patient (1), supporting considering use of anti-CD38 therapy without a plasma clone.