Immunoglobulin G4–Associated Cholangitis

New data suggest that IAC should be considered in the differential diagnosis for patients who have unexplained biliary strictures, unexplained pancreatic disease, and high levels of serum IgG4.

Immunoglobulin (Ig) G4–associated cholangitis (IAC) refers to the biliary manifestations of a steroid-responsive multisystem fibroinflammatory disorder, in which the involved organs have a characteristic lymphoplasmacytic infiltrate rich in IgG4-positive cells, and the serum contains high levels of IgG4. To better characterize this disease, researchers reviewed data from 53 patients with IAC; 49 (including 14 who had undergone pancreaticoduodenectomy for suspected pancreatic cancer) were identified from a database of patients with autoimmune pancreatitis (AIP). The remaining four patients were identified after they underwent bile-duct resection for suspected cholangiocarcinoma; all had characteristic histology for IAC but no evidence of AIP…

Reviewing Author

Stuart Sherman, MD