Recurrent liver failure caused by IgG4 associated cholangitis.

Area of Science:

• Gastroenterology and Hepatology
• Autoimmune Diseases
• Transplant Surgery

Background:

• Immunoglobulin G4 associated cholangitis (IAC) is an autoimmune condition often presenting similarly to primary sclerosing cholangitis (PSC).
• IAC typically exhibits a more rapid symptom onset and higher incidence of obstructive jaundice compared to PSC.
• Steroid therapy is a hallmark treatment for IAC, leading to disease remission.

Observation:

• A 59-year-old female with undiagnosed IAC developed liver failure after a pancreaticoduodenectomy for suspected cancer, initially presumed to be PSC.
• The patient underwent liver transplantation, but experienced allograft failure within five years due to progressive bile duct injury, with recurrent PSC suspected.
• Diagnosis of IAC was confirmed via liver biopsy showing IgG4 positive cells, despite initial radiology and histology suggesting PSC recurrence.

Findings:

• The patient underwent successful liver retransplantation and is currently on triple immunosuppressive therapy.
• Recurrent PSC with atypical features, including a history of pancreatitis, warrants testing for IAC.
• IAC is highly responsive to steroid therapy, suggesting its importance in management.

Implications:

• This case highlights the importance of considering IAC in patients with suspected or recurrent PSC, especially those with atypical presentations or a history of pancreatitis.
• Prompt diagnosis and initiation of steroid therapy for IAC can prevent disease progression and potentially avoid the need for retransplantation.
• Further research into differentiating IAC from PSC and optimizing treatment protocols is warranted.