The prevalence of primary biliary cholangitis (PBC) is up to 35/100,000 in US populations, with a distribution that is heavily skewed toward women (10:1 female-to-male distribution) and those over 45 years of age.
The combination of elevation of serum alkaline phosphatase and a PBC-specific autoantibody (typically antimitochondrial antibody) is sufficient for diagnosis in most patients, with no need for biopsy confirmation.
PBC is progressive in most patients; although, in many people, the rate of progression can be so slow that it may not be clinically relevant. Cirrhosis and its typical complications arise in the end stage.
Symptoms (typically pruritus and fatigue) can significantly lower the quality of life, even in patients with a very slowly progressive disease. These symptoms warrant treatment in their own right using specific regimens.
Progression of the disease can be slowed by therapy with ursodiol.
Obeticholic acid is recommended in patients with an inadequate response to ursodiol. The US Food and Drug Administration has restricted the use of obeticholic acid in patients with PBC with advanced cirrhosis because it can cause serious liver injury in this patient population.
Transplantation is an effective treatment for those patients who develop end-stage liver disease with PBC.
Primary biliary cholangitis (PBC) is a chronic disease of the small intrahepatic bile ducts that is characterized by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation. Fibrosis develops as a consequence of the original insult and the secondary effects of toxic bile acids retained in the liver, resulting ultimately in cirrhosis. The almost universal presence of autoantibodies in PBC patients (classically antimitochondrial antibodies) has led to the widely held view that the disease has an autoimmune component to its etiology.
History and exam
Key diagnostic factors
- age 45-60 years
- female sex
Other diagnostic factors
- personal history of autoimmune disease
- family history of autoimmune disease
- history of hypercholesterolemia
- dry eyes and dry mouth
- sleep disturbance
- postural dizziness/blackouts
- family history of primary biliary cholangitis
- weight loss and other metabolic features of malabsorption
- memory and concentration problems
- skin pigmentation
- female sex
- age between 45-60 years
- family history of PBC/autoimmune disease
- urinary tract infection
1st investigations to order
- alkaline phosphatase
- alanine aminotransferase (ALT)
- serum albumin
- antimitochondrial antibody immunofluorescence
- antinuclear antibody (ANA) immunofluorescence
- antipyruvate dehydrogenase complex-E2 ELISA
- anti-M2 ELISA
- antiglycoprotein-210 ELISA
- anti-Sp100 ELISA
- abdominal ultrasound scan
- magnetic resonance cholangiopancreatography
Investigations to consider
- prothrombin time
- serum immunoglobulin
- liver biopsy
- upper GI endoscopy
- serum alpha-fetoprotein
- transient elastography
developing end-stage liver disease or refractory pruritus
- Obstructive bile duct lesion
- Small-duct primary sclerosing cholangitis
- Drug-induced cholestasis
- Primary biliary cholangitis
- The diagnosis and management of patients with primary biliary cholangitis
MELDNa scores (for liver transplantation listing purposes, not appropriate for patients under age 12 years)More Calculators
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