Immunoglobulin G4–related disease of the gallbladder and the biliary tree

Abstract

Background: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a rare, immune-mediated fibroinflammatory disorder that can affect multiple organ systems, including the gallbladder and the biliary tree. This study aimed to characterize the clinical, imaging, and histopathologic features of IgG4-related biliary disease, including gallbladder involvement, and to review current medical and surgical management strategies. Methods: A comprehensive review of the literature was conducted using PubMed and Embase databases for studies published between 2003 and 2025. Inclusion criteria focused on English-language studies describing IgG4-RD affecting the gallbladder and biliary system, with detailed descriptions of diagnosis and treatment. Results: The most frequently reported symptoms included abdominal pain, jaundice, and fever. Common imaging findings were gallbladder wall thickening, pericholecystic fluid, and bile duct strictures. Histopathology showed lymphoplasmacytic infiltrates, storiform fibrosis, and IgG4-positive plasma cells. Most patients were treated successfully with corticosteroids, whereas a subset received rituximab for refractory or relapsing disease. Surgery is often performed for cases refractory to medical management or when malignancy cannot be ruled out. Conclusion: IgG4-RD of the biliary tract, including gallbladder involvement, presents a diagnostic challenge owing to its resemblance to malignancy and other inflammatory conditions. Accurate diagnosis hinges on integrating clinical, radiologic, and histologic findings. Medical therapy remains the mainstay of treatment, with surgery reserved for diagnostic uncertainty or refractory cases.