Abstract: TH-PO0724
Isolated IgG4-Related Tubulointerstitial Nephritis with Rapid Progression Requiring Hemodialysis
Session Information
- AKI: Prevention, Diagnostics, and Management
October 22, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 101 AKI: Epidemiology, Risk Factors, and Prevention
Authors
- Yos, Kimberly, St. Mary Medical Center, Long Beach, California, United States
- Kim, Jesper, St. Mary Medical Center, Long Beach, California, United States
- Lajevardi, Sherin, St. Mary Medical Center, Long Beach, California, United States
- Zaidi, Navid S., St. Mary Medical Center, Long Beach, California, United States
Introduction
IgG4-related kidney disease (IgG4-RKD) most commonly manifests as tubulointerstitial nephritis and is typically associated with multisystem IgG4-related disease involving organs such as the pancreas, salivary glands, or retroperitoneum. Renal-limited presentations without systemic manifestations are uncommon and often overlooked, and severe cases requiring dialysis are rarely described.
Case Description
A 45-year-old woman presented with progressive renal dysfunction. Her baseline creatinine was 0.89 mg/dL with an estimated glomerular filtration rate (eGFR) of 81 mL/min/1.73 in June 2025, which increased to 4.52 mg/dL with an eGFR of 12 by January 2026, ultimately requiring initiation of hemodialysis for renal failure and refractory volume overload. Her clinical course was complicated by recurrent pyelonephritis and hydronephrosis requiring nephrostomy tube placement. Persistent left sided hydronephrosis without a discrete obstructing lesion, along with worsening renal function, prompted renal biopsy. Pathology demonstrated chronic active tubulointerstitial nephritis with increased IgG4-positive plasma cells, multifocal fibro-expansile changes, and diffuse granular interstitial IgG staining. Serum IgG (2,027 mg/dL) and IgG4 (184 mg/dL) with polyclonal hypergammaglobulinemia on SPEP/IFE further supported the diagnosis of IgG4-RKD. The patient was treated with high-dose prednisone followed by rituximab therapy with subsequent improvement in renal function.
Discussion
In patients with unexplained or rapidly progressive renal failure despite plausible alternative etiologies, renal biopsy remains essential to establish the diagnosis. Early recognition of IgG4-related kidney disease is critical, as timely immunosuppressive therapy may allow for meaningful renal recovery even after dialysis initiation.
Renal biopsy demonstrating 25% global glomerulosclerosis (14/56 glomeruli; expected ≤7% for age) with severe tubulointerstitial fibrosis and tubular atrophy (60% of the renal parenchyma), consistent with IgG4-related tubulointerstitial nephritis.