[Autoimmune mediated cholestatic liver diseases]

Area of Science:

• Hepatology
• Autoimmune Diseases
• Gastroenterology

Background:

• Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are rare autoimmune cholestatic liver diseases.
• These conditions are frequently associated with other autoimmune disorders, and inflammatory bowel disease is common in PSC patients.
• Chronic liver inflammation in PBC and PSC can progress to fibrosis and cirrhosis.

Purpose of the Study:

• To provide an overview of the current understanding and management of PBC and PSC.
• To highlight diagnostic approaches and therapeutic strategies for these rare liver diseases.
• To discuss emerging treatments and the role of liver transplantation.

Main Methods:

• Diagnosis of PBC relies on serological markers.
• PSC diagnosis is confirmed via cholangiography, with MRCP preferred over ERCP.
• Review of current therapeutic guidelines and emerging clinical trials for PBC and PSC.

Main Results:

• Ursodeoxycholic acid (UDCA) is the first-line therapy for PBC, with prognosis dependent on response.
• Obeticholic acid (OCA) is the only approved second-line therapy for PBC; budesonide or fibrates are used off-label.
• Management of PSC focuses on preventing and treating bacterial cholangitis; novel treatments are under investigation for both diseases.

Conclusions:

• Effective management of PBC and PSC requires addressing associated symptoms like pruritus and sicca, and complications such as maldigestion and other autoimmune diseases.
• Liver transplantation is the only curative treatment for end-stage liver disease, recommended at a MELD score of 15.
• Ongoing clinical trials are exploring novel therapeutic options for both PBC and PSC, aiming to improve patient outcomes.