Immunoglobulin G4-Seronegative Autoimmune Cholangiopathy With Pancreatic and Hepatic Involvement Mimicking as Primary Sclerosing Cholangitis

Sudharshan Achalu; Rani Berry; Mike T Wei; Subhas Banerjee; Pejman Ghanouni; Neeraja Kambham; Paul Y Kwo

doi:10.14309/crj.0000000000001044

Immunoglobulin G4-Seronegative Autoimmune Cholangiopathy With Pancreatic and Hepatic Involvement Mimicking as Primary Sclerosing Cholangitis

ACG Case Rep J. 2023 Apr 20;10(4):e01044. doi: 10.14309/crj.0000000000001044. eCollection 2023 Apr.

Authors

Sudharshan Achalu¹, Rani Berry¹, Mike T Wei¹, Subhas Banerjee¹, Pejman Ghanouni², Neeraja Kambham³, Paul Y Kwo¹

Affiliations

¹ Department of Medicine, Division of Gastroenterology and Hepatology, Stanford University School of Medicine, Redwood City, CA.
² Department of Radiology, Stanford University School of Medicine, Palo Alto, CA.
³ Department of Pathology, Stanford University School of Medicine, Palo Alto, CA.

Abstract

Immunoglobulin G4-seronegative autoimmune cholangiopathy is a rare cause of biliary strictures. We describe a 27-year-old man presenting with elevated liver enzymes, recurrent cholangitis/bacteremia, biliary strictures, and normal immunoglobulin G4 levels, who was initially diagnosed with primary sclerosing cholangitis, and later listed for transplantation for recurrent bacteremia. Subsequent surveillance imaging demonstrated morphologic changes consistent with biliary strictures and autoimmune pancreatitis. Initiating corticosteroids resulted in liver enzyme normalization and stricture improvement. Diagnosing seronegative autoimmune cholangiopathy remains challenging given similar presentation to primary sclerosing cholangitis. This case highlights importance of a wide differential for biliary strictures, with increased suspicion in those developing pancreatic changes in this setting.

Keywords: IgG4 cholangiopathy; PSC; autoimmune pancreatitis; biliary strictures; primary sclerosing cholangitis.

Publication types

Case Reports