Abstract: SA-PO0901
IgG4-Related Tubulointerstitial Nephritis and Proliferative Glomerulonephritis (GN) with IgG2, IgG3, and IgG4 Deposits in a Patient with Treated T Cell-Rich Large B Cell Lymphoma
Session Information
- Glomerular Case Reports: ANCA, IgA, IgG, and More
November 08, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Oh, Jaha, Weill Cornell Medicine, New York, New York, United States
- Seshan, Surya V., Weill Cornell Medicine, New York, New York, United States
- Latcha, Sheron, Memorial Sloan Kettering Cancer Center, New York, New York, United States
Introduction
IgG4-related kidney disease (IgG4-RKD) most commonly presents as tubulointerstitial nephritis (TIN) with occasional glomerular involvement. Recent literature suggests an association between IgG4-RKD and hematologic malignancies. We report a unique case of IgG4-TIN with concurrent immune complex mediated proliferative GN in a patient with prior treated lymphoma.
Case Description
A 77-year-old man with stage 3a CKD and history of nodular lymphocyte-predominant Hodgkin lymphoma (treated with R-CHOP, DICE, and autologous stem cell transplant, in remission) presented with AKI and petechial rash. Following recent travel to Mexico, he developed spreading petechial rash, joint pains and fevers. Laboratory testing showed elevated BUN, creatinine (3.5 mg/dL, baseline 1.4), mild anemia, thrombocytopenia (55K), proteinuria, low C3 and C4. Imaging revealed new lymphadenopathy.
Skin biopsy showed urticarial dermatitis; biopsy from right inguinal lymph node showed chronic inflammation without lymphoma. Kidney biopsy revealed a dominant IgG4-positive plasma cell-rich interstitial infiltrate consistent with IgG4-TIN, and diffuse proliferative GN with polyclonal IgG, including IgG2, IgG3 and dominant IgG4 immune deposits (Figure 1, 2). No evidence of thrombotic microangiopathy or lymphoid malignancy was found. Extensive rheumatologic and infectious workup was negative.
High-dose corticosteroids led to rapid improvement in renal function and resolution of symptoms. AKI recurred upon rapid steroid taper and improved with re-initiation of steroid.
Discussion
This case highlights the rare coexistence of IgG4-TIN and proliferative GN with polyclonal IgG immune deposits in a patient with prior large B-cell lymphoma. While IgG4-RD is more commonly associated with membranous nephropathy, proliferative GN with MPGN-like features remains rare. The temporal association with treated lymphoma raises consideration for a paraneoplastic or immune reconstitution-related phenomenon. Early steroid therapy was associated with rapid clinical response.