Internal Medicine

0521779407-17 CUNY1086/Karliner 0 521 77940 7 June 13, 2007 7:57

Primary Biliary Cirrhosis 1209

PRIMARY BILIARY CIRRHOSIS

ANDY S. YU, MD and JOANNE C. IMPERIAL, MD

history & physical

History

■primarily affects middle-aged women (median age 50 at diagnosis)

■most cases are diagnosed while asymptomatic with incidental serum

alkaline phosphatase elevation

■fatigue: most common symptom

■pruritus: most specific symptom

■association with extrahepatic diseases: arthritis (20%), sicca syn-

drome (70%), scleroderma/CREST (15%), thyroiditis (10–15%), type

I & II renal tubular acidosis (50–60%), celiac disease (6%)

Physical Signs

■early disease: hyperpigmentation, hepatomegaly, splenomegaly,

jaundice, xanthelasma

■advanced disease: liver failure

tests

Laboratory

Basic Studies: Blood

■early disease

➣alkaline phosphatase at least 3–4 x normal, increased GGT,

increased IgM (95% of cases), increased serum cholesterol level,

increased AST and ALT

■advanced disease

➣increased serum bilirubin and INR, decreased serum albumin

■serology

➣positive AMA≥1:40; M2 antibody (highly sensitive and specific)

➣positive SMA, antithyroid antibody, antimicrosomal and antithy-

roglobulin antibodies, ANA, rheumatoid factor are nonspecific

Liver Biopsy

■may not be necessary if clinical, biochemical, and serological fea-

tures are all typical of PBC; useful to exclude other causes of liver

disease

■early disease: inflammatory destruction of intrahepatic bile ducts

granulomatous destruction of bile ducts (florid duct lesion), prolif-

eration of bile ductules

■advanced disease: scarring with regenerative nodules