[Hepatic Overlap Syndrome: Primary Sclerosing Cholangitis and Primary Biliary Cholangitis. Case Review]

Area of Science:

• Hepatology
• Gastroenterology
• Internal Medicine

Background:

• The overlap between primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) is an exceptionally rare liver disease association.
• This PBC-PSC overlap syndrome is the least prevalent among liver disease overlap syndromes, including those with autoimmune hepatitis (AIH).

Purpose of the Study:

• To review and analyze clinical cases of patients diagnosed with PBC-PSC overlap syndrome at the Hospital Clínico de la Universidad de Chile.
• To characterize the clinical presentation and diagnostic findings of this rare condition.

Main Methods:

• A descriptive observational study was conducted.
• Clinical records of patients with PBC and PSC overlap syndrome between 2021 and 2023 were reviewed.

Main Results:

• Eight patients with PBC-PSC overlap syndrome were identified, all of whom were women.
• Patients exhibited cholestatic liver function tests and positive anti-mitochondrial (AMA) antibodies.
• Radiographic imaging revealed focal stenosis consistent with PSC, with one case classified as small duct PSC.

Conclusions:

• Despite its rarity, diagnosing PBC-PSC overlap syndrome is clinically significant.
• Distinct treatment strategies and targeted screening for complications are necessary for patients with this overlap syndrome.