P3104 - The Silent Saboteur: A Case of IgG4-Predominant Plasma Cell-Rich Rejection (PCR) in a Liver Transplant Recipient

Owais N. Syed, MD¹, Courtney Thomas, DO¹, Eugenia Tsai, MD¹, Lisa Pedicone, PhD², Fabian Rodas, MD³, Carmen Landaverde, MD⁴, Andres Gomez-Aldana, MD², Eric Lawitz, MD¹, Fred Poordad, MD⁵, Jan Petrasek, MD, PhD^2
1University of Texas Health San Antonio, San Antonio, TX; ²Texas Liver Institute, San Antonio, TX; ³The Texas Liver Institute, San Antonio, TX; ⁴Texas Liver Institute, Austin, TX; ⁵UTHSCSA, Texas Liver Institute, San Antonio, TX

Introduction: Plasma cell-rich rejection (PCR) is an uncommon but potentially devastating cause of late liver allograft dysfunction. We present a challenging case of steroid-resistant IgG4-predominant PCR in a liver transplant recipient.

Case Description/Methods: A 48-year-old female developed obstructive jaundice seven months post-liver transplant for alcohol-associated cirrhosis. Bilirubin was 19.9 mg/dL and alkaline phosphatase was 1034 U/L. ERCP revealed multiple non-anastomotic inflammatory biliary strictures. A liver biopsy demonstrated extensive portal and lobular plasmacytic inflammation (Figure 1a) and cholestasis suggesting a major duct obstruction. Immunohistochemical staining (Figure 1b) and elevated serum IgG4 (1769 mg/dL) confirmed IgG4-predominant PCR. Subsequent IgG4 levels at the one-month, two-month, three-month and four-month post treatment initiation improved to 1245 mg/dL, 687 mg/dL, 595 mg/dL and 463 mg/dL respectively. Despite initial treatment with high-dose IV steroids and tacrolimus, repeat ERCP showed persistent strictures, and a repeat biopsy four months later revealed persistent plasmacytic inflammation and stage 2 fibrosis. Treatment with anti-CD20 therapy and plasma exchange were deferred due to concurrent bacteremia at the time of repeat biopsy but are considered after the resolution of bacteremia.

Discussion: PCR, a rare but significant complication after liver transplantation, is characterized by dense plasma cell-rich infiltrates in the absence of prior autoimmune liver disease. While the rare IgG4-predominant subtype, may respond to steroids, this case highlights the potential for discordance between improved IgG4 levels and clinical or histological improvement, even with steroid therapy. This underscores the need for further research to optimize treatment strategies, particularly in steroid-resistant cases. Given the poor prognosis and high risk of graft loss associated with PCR, early recognition and aggressive treatment are paramount to preserving allograft function.



Disclosures:


Owais N. Syed, MD¹, Courtney Thomas, DO¹, Eugenia Tsai, MD¹, Lisa Pedicone, PhD², Fabian Rodas, MD³, Carmen Landaverde, MD⁴, Andres Gomez-Aldana, MD², Eric Lawitz, MD¹, Fred Poordad, MD⁵, Jan Petrasek, MD, PhD². P3104 - The Silent Saboteur: A Case of IgG4-Predominant Plasma Cell-Rich Rejection (PCR) in a Liver Transplant Recipient, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.