Primary Sclerosing Cholangitis (PSC)
Epidemiology:
- Typically adults
with UC
- 3rd to 5th decades
- also neonates
and children
- M > F (2:1)
Common sites:
- Any part of the biliary tree may be affected
- Gallbladder and pancreas
involvement has been reported
Gross features:
Histologic features:
- disease of large bile
ducts primarily
- concentric periductal edematous fibrosis (“onion-skin fibrosis”)
- progresses to
disappearance of duct, leaving a solid, cordlike fibrous scar
- ductular proliferation
- portal inflammation
- loss of ducts in
smallest portal tracts
- in-between areas
of stricture:
- major ducts
inflamed, ulcerated, or dilated
- PASD staining shows
thickening of basement membrane around scarred and unscarred ducts
- cholestatic changes as progresses
- biliary cirrhosis at end-stage
- obliteration or narrowing of hepatic arteries and
portal vein branches (drug-related cases only?)
- may see only cholestasis if biopsy from an uninvolved proximal
area
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- characteristic
“beading” on ERCP (intrahepatic and extrahepatic)
- commonly in association
with IBD, particularly UC (UC in 70% of PSC patients) (PSC in 4% of UC patients)
- autoantibodies in less than 10%
- protracted course
over many years
- increased risk for
cholangiocarcinoma
- anti-cancer drug fluorodeoxyuridine (FUDR) and other chemotherapeutic
agents may produce lesions similar to PSC
- no effective
treatment
- liver transplant
is treatment of choice
- complications:
- fat-soluble
vitamin deficiencies
- metabolic bone disease
- recurrent
bacterial cholangitis
- cholelithiasis
- bile duct
strictures
- cholangiocarcinoma (10-15%)
References:
- Robbins 2005
- Scheuer and Lefkowitch
2000
- Essentials of AP 2006