Clinical Updates in IgG4-Related Pancreatic and Hepatobiliary Disease

Journal article

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory immune-mediated condition. The Pancreato-Hepato-Biliary subtype is the most frequent phenotype. The subtype encompasses immunoglobulin G4 (IgG4)-related pancreatitis (autoimmune pancreatitis type 1), IgG4-related cholangitis, IgG4-related hepatopathy and IgG4-related cholecystitis. The condition is characterised by multi-organ involvement and unpredictable recurrent disease flares, which lead to organ damage and failure in the absence of early recognition and adequate treatment. It is associated with a 2-fold increase in mortality and higher incidences of malignancies, such as solid-organ tumours and lymphoma. Diagnostic delays result from the lack of awareness and the absence of a single diagnostic test, leading to multiple generalist and specialist visits prior to the initiation of therapy. There have been major advances in the management of IgG4-RD to include a move to more targeted therapies. This includes the novel cluster of differentiation 19 (CD19)-depletion therapy inebilizumab, which has been approved in the United States, Europe and Japan for the treatment of IgG4-RD. This review highlights crucial diagnostic clues and emerging therapeutic advances in the evolving field of the Pancreato-Hepato-Biliary subtype of IgG4-RD.

Authors: Lee, H; Motta, RV; Culver, EL

• Publication status: Published
• Peer review status: Peer reviewed
• Publisher: MA Healthcare
• Journal: British Journal of Hospital Medicine
• Volume: 87
• Issue: 6
• Pages: 55132-55132
• Publication date: 2026-06-22
• Acceptance date: 2025-12-22
• DOI: 10.31083/bjhm55132
• EISSN: 1759-7390
• ISSN: 0007-1064, 1750-8460
• Language: English
• Keywords: Pancreatitis; Disease; Autoimmune pancreatitis; Pancreatic disease; Antibody; Multisystem disease; Acute pancreatitis