Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap in a Case of Pulmonary Arterial Hypertension and Sjögren's Syndrome: Occam's Razor or Hickam's Dictum?

TL;DR

A 37-year-old woman with right heart failure and Sjögren's syndrome was found to have an overlap of autoimmune hepatitis and primary biliary cholangitis after persistent liver abnormalities.

Contribution

This case highlights the importance of considering autoimmune liver diseases in patients with persistent liver dysfunction despite resolving heart failure.

Findings

The patient's liver function tests remained abnormal despite resolution of heart failure symptoms.

Liver biopsy and antibody testing confirmed an overlap of autoimmune hepatitis and primary biliary cholangitis.

Treatment with prednisolone, azathioprine, and ursodeoxycholic acid improved liver function and overall status.

Abstract

Right-sided heart failure can lead to abnormalities in liver function tests due to hepatic congestion, a condition known as congestive hepatitis. This condition typically responds to diuretics, as well as salt and fluid restriction. However, it is crucial to consider other potential causes of liver function abnormalities if they persist despite optimal therapeutic interventions, to avoid missing treatable conditions.

I present the case of a 37-year-old woman with right heart failure secondary to pulmonary hypertension and Sjögren's syndrome. Initially, her liver function test abnormalities were attributed to congestive hepatitis. However, during follow-up, it was noted that despite the resolution of her ascites, pleural effusion, and pedal edema, her liver function tests remained abnormal. This persistence prompted an investigation for an alternative cause.

Subsequent testing revealed the presence of anti-nuclear antibodies, anti-mitochondrial antibody (AMA) positivity, and speckled protein 100 (Sp100) positivity, as well as elevated immunoglobulin G (IgG) levels. Additionally, a liver biopsy demonstrated interface hepatitis, lymphoplasmacytic infiltrates, and the destruction of medium-sized bile ducts. These findings were consistent with an overlap of autoimmune hepatitis and primary biliary cholangitis.

She was started on prednisolone, azathioprine, and ursodeoxycholic acid. On follow-up, her liver function tests gradually returned to baseline, and her functional status improved.

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