Abstract: SA-PO1216
De Novo IgG-Dominant Immune-Complex Glomerulopathy After Successful Dual Kidney-Liver Transplantation in Alagille Syndrome
Session Information
- Transplantation: Clinical - Complications, Pediatrics, and Multi-Organ Considerations
October 24, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Transplantation
- 2002 Transplantation: Clinical
Authors
- Khan, Muhammad Ulusyar, Washington University in St Louis, St. Louis, Missouri, United States
- Alrata, Louai, Washington University in St Louis, St. Louis, Missouri, United States
- Merzkani, Massini, Washington University in St Louis, St. Louis, Missouri, United States
- Alhamad, Tarek, Washington University in St Louis, St. Louis, Missouri, United States
Introduction
Alagille syndrome(ALGS) is a rare autosomal dominant multisystem disorder commonly caused by JAG-1 mutations.Renal involvement contributes to 39% of patients;however,progression to end-stage kidney disease(ESKD) and the need for multi-organ transplantation are uncommon. Mesangial Immune Complex (IC) injury, particularly due to IgG in ALGS has been rarely reported
Case Description
A 16-Year-old male with known ALGS due to JAG-1 mutation underwent liver transplant at age 1 for hepatic failure, later complicated by graft failure and development of IgA type IC glomerulopathy and calcineurin inhibitor nephrotoxicity, leading to ESKD. He subsequently received a successful dual kidney-liver transplant (DKLT) 10 years(yrs) post-first liver transplant. Laboratory follow-up after 2 yrs of DKLT revealed mild allograft dysfunction (Cr 1.52 mg/dL) with minimal proteinuria(<200mg/g) and normal urine sediment. Kidney allograft biopsy revealed de novo mesangial IgG-dominant IC glomerulopathy. Immunofluorescence showed granular deposits with IgG (1-2 +), C3 (3+), C1q (3+), λ(2+), κ (2+), and C4d (3+). Numerous electron-dense deposits with normal Glomerular basement membrane thickness and no subendothelial expansion on electron microscopy. Banff class 2 Polyomavirus Nephritis with severe tubulitis(t3), interstitial inflammation(i3), and severe peritubular capillaritis(ptc3).Figure 1
Tacrolimus was reduced and mycophenolate discontinued.The patient showed stable renal function and BK viral load four years post-DKLT.
Discussion
Despite concurrent BK virus nephropathy, the lupus-like immunofluorescence pattern with negative autoimmune serologies and stable clinical course favored a distinct immune-mediated process rather than BK or Lupus driven glomerulonepathy. This association has not previously been reported and highlights the importance of integrating histological, virologic, and immunological findings in complex transplant recipients.
Histological findings