Primary Biliary Cholangitis

Primary Biliary Cholangitis is a topic covered in the Washington Manual of Medical Therapeutics.

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General Principles

Primary biliary cholangitis (PBC) is a cholestatic hepatic disorder of unknown etiology with autoimmune features. It is formerly known as primary biliary cirrhosis

  • It most often affects middle-aged women (>90%) and is more commonly described in Caucasians. It is caused by granulomatous destruction of the interlobular bile ducts, which leads to progressive ductopenia and cholestasis.
  • Cholestasis is generally slowly progressive and can lead to cirrhosis and liver failure.
  • Extrahepatic manifestations include keratoconjunctivitis sicca (Sjögren), renal tubular acidosis, gallstones, thyroid disease, scleroderma, Raynaud phenomenon, CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia), and celiac disease.

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