Gastrointestinal Imaging

Autoimmune Disorders of the Liver and Biliary Tract

Published Online:Mar 20 2025https://doi.org/10.1148/rg.240126

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Abstract

Understanding the imaging appearance of autoimmune disorders of the liver and biliary tract is important to guide further workup, suggest a particular diagnosis, monitor for disease progression or response to treatment, and evaluate for complications.

Several autoimmune diseases (primary and secondary) can affect the liver and bile ducts. While the exact cause remains unclear, early diagnosis is crucial to prevent complications. The authors’ main objective is to review imaging features of various autoimmune disorders, including autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, immunoglobulin G4 (IgG4)–related diseases, and drug-induced autoimmune injury. These disorders are chronic inflammatory conditions causing destruction of hepatocytes or cholangiocytes, destruction of the latter potentially leading to cholestasis and associated ductopenia. Complications related to untreated autoimmune disorders include sequelae of chronic liver failure or cirrhosis, such as portal hypertension and ascites. Neoplasms arising in the setting of cirrhosis related to autoimmune diseases include hepatocellular carcinoma, cholangiocarcinoma, and gallbladder cancer. As these autoimmune disorders of the liver and biliary tract characteristically involve bile ducts and cause cholestasis, MRI or MR cholangiopancreatography (MRCP) is the preferred imaging modality, given its ability to provide excellent anatomic details of the bile ducts and demonstrate changes in the liver parenchyma. Understanding the imaging appearance of each of the autoimmune disorders affecting the liver and biliary tract allows a particular diagnosis to be suggested. Imaging studies often provide the first clues to an autoimmune disorder of the liver and bile ducts, enabling early diagnosis to halt progression and prevent complications. In addition, imaging studies are also useful for monitoring progression of disease, assessing treatment response, and detecting complications during follow-up.

^©RSNA, 2025

An earlier incorrect version of this article appeared online. This article was corrected on March 25, 2025.

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Article History

Received: Apr 20 2024
Revision requested: May 21 2024
Revision received: July 1 2024
Accepted: July 3 2024
Published online: Mar 20 2025

Vol. 45, No. 4

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Abbreviations

Abbreviations:
AIH	autoimmune hepatitis
HCC	hepatocellular carcinoma
IgG4	immunoglobulin G4
IgG4-RD	IgG4-related disease
IgG4-SC	IgG4-related sclerosing cholangitis
MIP	maximum intensity projection
MRCP	MR cholangiopancreatography
NRH	nodular regenerative hyperplasia
PBC	primary biliary cholangitis
PSC	primary sclerosing cholangitis

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