Primary Biliary Cirrhosis (PBC)
Epidemiology:
- Middle-aged women
- Female > male
(6:1)
Common sites:
Gross features:
- hepatomegaly typical
- jaundiced over
time
- capsule normal to
fine granular to uniform micronodularity
- liver weight at first
normal to increased, then slightly decreased
Histologic features:
- non-suppurative inflammatory destruction of medium-sized intrahepatic bile ducts
- focal, variable
within liver
- precirrhotic stage:
- dense periportal lymphocytes, macrophages, plasma cells,
occasional eos
- terminal and
conducting bile ducts infiltrated by lymphocytes
- may
have noncaseating granulomatous
inflammation
- progressive
destruction of these ducts
- progressive
secondary cholestasis and resulting hepatic
damage
- portal tracts
upstream from damaged bile ducts show bile ductular
proliferation, inflammation, and necrosis of adjacent periportal hepatic parenchyma
- over years to
decades:
- portal tract
scarring
- bridging
fibrosis
- 4 stages:
- the florid duct
lesion; portal hepatitis
- involves
septal and larger interlobular ducts mainly
- ductal
epithelium is irregular and infiltrated with lymphocytes
- basement
membrane disrupted
- duct
may rupture
- inflammatory
infiltrate around duct
- mainly
lymphocytes and plasma cells
- also
eosinophils and neutrophils
- may
form aggregates or follicles with germinal centres
- granulomas often
- or
small focal collections of histiocytoid
cells
- usually
in portal areas but sometimes intra-acinar
- ductular proliferation and periportal hepatitis
- associated
with an infiltrate of neutrophils
- scarring; bridging
necrosis, septal fibrosis
- extends
beyond the portal tracts
- fibrosis
- alteration
of acinar architecture
- bile
duct damage less dramatic
- but
progressive fall in numbers (compare to number of arteries)
- granulomas
fewer
- portal
tracts expand progressively
- cholestasis
- lymphocytes
bridging into acini
- increased
intrahepatic mast cells
- cirrhosis
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- autoimmune
- Extrahepatic autoimmunity associated (Sjogren, scleroderma, thyroiditis,
RA, Raynaud, membranous glomerulonephritis,
celiac)
- Antimitochondrial antibodies in 90%
- insidious course
- cirrhosis happens
only after many years
- xanthomas, xanthelasmas
(cholesterol retention)
- ALP and
cholesterol almost always elevated
References:
- Robbins 2005
- Scheuer & Lefkowitch
2000