Primary biliary cholangitis

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune chronic liver disease characterised by progressive destruction of intralobular bile ducts leading to intrahepatic cholestasis. Eventually, cirrhosis develops.

It almost exclusively affects middle-aged women and is associated with other autoimmune disorders.

Clinical features

PBC may present asymptomatically (only abnormal laboratory parameters) as it remains asymptomatic for a long time. Then, nonspecific symptoms like fatigue and pruritus appear. Later, symptoms of cholestasis appear, like jaundice, itching, and xanthelasmas.

Diagnosis and evaluation

As with any cholestasis, ALP, GGT, and bilirubin are elevated. However, the following findings are typical for PBC:

• Autoantibodies (ANA, AMA)
• Hyperlipidaemia
• Mildly increased AST and ALT

Symptoms of cholestasis often prompts morphological examination of bile ducts with ERCP or MRCP, but these are normal in case of PBC. Ultrasound can also be used to in the evaluation

Biopsy and histology are not necessary for the diagnosis but may give information on stage and prognosis. It will show destruction of the interlobular bile ducts.

Treatment

There is no cure for PBC, and so treatment aims at slowing disease progression and improving symptoms.

Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid which stimulates bile secretion and has a hepatoprotective effect and is used in the treatment of PBC. It slows the progression and improves the symptoms. Obetichoic acid is another option.

Cholestatic pruritus is a common and possibly disabling symptom. UDCA improves it, but bile acid sequestrants like cholestyramine or rifampin may also help.

Liver transplantation is the only definitive treatment but is reserved for end-stage disease or intractable pruritus.