Abstract
Purpose of review
Primary biliary cholangitis is a progressive autoimmune cholestatic liver disease more commonly affecting middle-aged women. Here we provide an overview of the diagnosis, clinical presentation, management, and prognosis of patients with PBC.
Recent findings
Ursodeoxycholic acid (UDCA) remains the first-line treatment for patients with PBC. Incomplete responders are at risk for disease progression. Obeticholic acid (OCA) is a second-line therapy that has shown benefit in patients with PBC who do not respond to or are intolerant of UDCA, and the off-label use of fibrates continues to show promise, including when used in combination with UDCA and OCA. Newer therapies are under investigation.
Summary
Early diagnosis of PBC combined with the initiation of UDCA remains essential to prevent end-stage liver disease. Risk-stratifying after 1 year of treatment and the need for adjuvant therapy is important to prolong transplant-free survival time.