Primary biliary cholangitis

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Synonyms

PBC, primary biliary cirrhosis,

Overview

Symptoms

Primary Biliary Cholangitis (PBC) often presents with no symptoms initially, but commonly causes severe fatigue and intense, often nocturnal, skin itching (pruritus). Other frequent symptoms include dry eyes/mouth, upper right abdominal discomfort, and joint pain. As the condition progresses, it may cause jaundice, fat-soluble vitamin deficiency, and fluid buildup.

Causes

While the exact trigger is unknown, it is believed to result from a combination of genetic predisposition and environmental factors like infections or chemicals, which activate immune cells to cause bile duct damage, leading to inflammation, fibrosis, and potentially cirrhosis.

Prevention

Primary Biliary Cholangitis (PBC) cannot be prevented because its exact cause is unknown, but its progression can be slowed by managing liver health, taking ursodeoxycholic acid (UDCA), and maintaining a healthy lifestyle. Key measures include stopping smoking, limiting alcohol, reducing sodium and saturated fats, exercising regularly, and avoiding raw shellfish.

Diagnosis

PBC is diagnosed through blood tests showing elevated alkaline phosphatase (ALP) and the presence of antimitochondrial antibodies (AMA), often confirmed without biopsy if these markers are present. Imaging (ultrasound, MRCP) is used to rule out bile duct obstruction, while liver biopsy is reserved for cases with negative antibodies.

Prognosis

Primary Biliary Cholangitis (PBC) is a chronic, slow-progressing autoimmune liver disease with a generally good prognosis when treated early with medications like ursodeoxycholic acid (UDCA/Ursodiol). While incurable, early treatment can allow patients to live a near-normal life expectancy. Advanced, untreated cases can lead to liver failure or necessitate transplant.

Treatment

Primary Biliary Cholangitis (PBC) is primarily treated with Ursodeoxycholic acid (UDCA or Urso) to slow liver damage, often for life. Second-line therapies for non-responders include obeticholic acid, fibrates, or new PPAR agonists (seladelpar, elafibranor). While not curable with medicine alone, progression can be managed, with liver transplant as a final option for severe failure.