Primary Biliary Cirrhosis



Primary Biliary Cirrhosis


Matthew M. Yeh, MD, PhD





Key Facts


Terminology



  • Idiopathic chronic cholestatic autoimmune liver disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation


Clinical Issues



  • Middle-aged to elderly women


  • Insidious onset with pruritus


  • Fatigue, jaundice, associated autoimmune disorders


  • Positive antimitochondrial antibodies (AMA)



    • Minority of cases are AMA(-)


  • Elevation of GGT, alkaline phosphatase, bilirubin


  • Ursodeoxycholic acid (UDCA) is treatment of choice


Microscopic Pathology



  • Florid duct lesion


  • Bile duct injury


  • Bile ductular reaction


  • Chronic cholestasis


  • Portal-based fibrosis


  • Copper stain highlights accumulated copper in hepatocytes (copper is normally excreted in bile)


Top Differential Diagnoses



  • Primary sclerosing cholangitis (PSC)


  • Secondary biliary obstruction


  • Drug-induced chronic cholestasis


  • Sarcoidosis


  • Autoimmune hepatitis



    • Some patients have autoimmune hepatitis-PBC overlap syndrome with features of both






The florid duct lesion with nonnecrotizing granulomas image is typical of primary biliary cirrhosis.






Primary biliary cirrhosis may require liver transplantation. This cholestatic explanted liver is from a patient with primary biliary cirrhosis.


TERMINOLOGY


Abbreviations



  • Primary biliary cirrhosis (PBC)


Definitions



  • Chronic cholestatic autoimmune disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation


ETIOLOGY/PATHOGENESIS


Unknown



  • Most likely multifactorial


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Up to 30/100,000 in Scandinavia and parts of North America



      • Much more prevalent among individuals of North European descent


    • Distinctly rare in Asians


  • Age



    • Middle-aged to elderly (40-60 years old)


  • Gender



    • Predominantly women (F:M = 9:1)


Presentation



  • Insidious onset with pruritus (most common), fatigue, jaundice, associated autoimmune disorders


Laboratory Tests



  • Positive antimitochondrial antibodies (AMA)


  • Elevation of GGT &/or alkaline phosphatase


  • Elevated bilirubin


  • Elevated IgM


  • Mildly elevated transaminases


Treatment



  • Surgical approaches



    • Liver transplantation


  • Drugs



    • Ursodeoxycholic acid (UDCA)


Prognosis



  • Chronic, progressive disease

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Jul 7, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Primary Biliary Cirrhosis

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