Unusual Case of Autoimmune Hepatitis Plus Primary Biliary Cholangitis With Atypical Liver Cancer

By: Julia Fiederlein Cipriano, MS
Posted: Monday, September 18, 2023

The overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) has been found to be less associated with the development of liver cirrhosis and subsequent hepatocellular carcinoma than other chronic liver diseases, especially in the absence of other risk factors. However, an unusual case of PBC-AIH overlap syndrome complicated with atypical hepatocellular carcinoma, which was described by Dan L. Dumitrașcu, MD, PhD, of the County Emergency Hospital, Cluj-Napoca, Romania, and colleagues in the Journal of Medical Case Reports, highlighted the importance of regular abdominal ultrasonography and alpha-fetoprotein monitoring in patients with cirrhosis for early cancer detection.

“PBC is an uncommon cholestatic [and] chronic autoimmune disease,” the investigators explained. “PBC-AIH overlap syndrome is diagnosed in about 8% to 10% of the patients initially diagnosed with PBC.”

A 71-year-old woman was previously diagnosed with overlap syndrome (autoimmune hepatitis type 1 and primary biliary cholangitis; antinuclear, smooth muscle, and antimitochondrial antibody–positive), liver cirrhosis, a 9- to 10-mm nodule in the VI/VII hepatic segment, gastric antral vascular ectasia, arterial hypertension, nephroangiosclerosis, and several autoimmune disorders. Her surgical and recent therapeutic histories included cholecystectomy, ursodeoxycholic acid, azathioprine, hydroxychloroquine, calcium channel blockers, and angiotensin-converting enzyme inhibition, as well as calcium and vitamin D supplementation.

During a follow-up examination, an abdominal ultrasound revealed a 29-mm subcapsular hypoechoic nodule in the VI/VII hepatic segment. Based on a contrast-enhanced ultrasound, it was categorized as a Liver Imaging Reporting and Data System (LI-RADS) category 5 lesion. In light of an alpha-fetoprotein level of 10.8 ng/mL, the investigators opted to conduct a gadoxetate disodium–enhanced MRI evaluation; the nodule exhibited hypovascularity and nonspecific characteristics, aligning with an LI-RADS 3 classification. An atypical resection of the VII hepatic segment was performed; histologic examination and immunohistochemistry revealed a moderately differentiated grade 2, pT2, N0 M0 L0 V1 R0 hepatocellular carcinoma.

Disclosure: The study authors reported no conflicts of interest.

Journal of Medical Case Reports

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