Primary Biliary Cirrhosis (PBC)



Primary Biliary Cirrhosis (PBC)





(NEJM 2005;353:1261-73. Hepatology. 2000;31:1005-13)


DEFINITION:



  • PBC: Autoimmune destruction of intrahepatic bile ducts (small bile ducts)


  • Autoimmune Cholangitis or AMA-Negative PBC: (may see positive ANA and/or ASMA)



    • Patients with PBC and non-detectable serum AMA; Response to therapy is the same as PBC


  • Overlap or Variant Syndrome: presence of features of both Autoimmune Hepatitis (AIH) and PBC



    • Positive ANA and AMA; Piecemeal necrosis and coexistent periductal/portal inflammation with bile duct destruction


EPIDEMIOLOGY:



  • Middle-aged women (age 40-60) occurring in 150-300 per million persons


  • Concomitant autoimmune disease (See also Pathophysiology section below)


  • Can see familial clustering


ETIOLOGIES:



  • A chronic cholestatic liver disease of unknown etiology


PATHOPHYSIOLOGY:



  • Autoimmune destruction of interlobular and septal (intrahepatic) bile ducts


  • Frequent association with other autoimmune diseases:



    • Sicca syndrome, RA, Hypothyroidism, Sjogren’s, Scleroderma, CREST, Dermatomyositis, MCTD, SLE, Lichen planus, Pemphigoid


  • DDX: Other causes of chronic cholestasis:



    • Biliary stones, Iatrogenic strictures (i.e. biliary surgery), Tumors; Drug-induced: phenothiazines, estrogens, etc.

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Aug 24, 2016 | Posted by in GASTROENTEROLOGY | Comments Off on Primary Biliary Cirrhosis (PBC)

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