Primary sclerosing cholangitis: Difference between revisions
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*Generally asymptomatic but can present with fatigue, abd pain, jaundice, cholangitis, puritis, weight loss, or fever | *Generally asymptomatic but can present with fatigue, abd pain, jaundice, cholangitis, puritis, weight loss, or fever | ||
*Mean age at presentation: 30-40 | *Mean age at presentation: 30-40 | ||
==Differential Diagnosis== | |||
==Diagnosis== | ==Diagnosis== | ||
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*Liver biopsy typically shows pericholangitis and periductual fibrosis but is often not diagnostic in early dz | *Liver biopsy typically shows pericholangitis and periductual fibrosis but is often not diagnostic in early dz | ||
== Work-Up == | === Work-Up === | ||
*Alkaline phosphatase is usually elevated with mild elevations in aminotransferases | *Alkaline phosphatase is usually elevated with mild elevations in aminotransferases | ||
*Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved in late stages of dz | *Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved in late stages of dz | ||
*Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases | *Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases | ||
== Treatment == | == Treatment == | ||
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== See Also == | == See Also == | ||
== | ==References== | ||
Current Clinical Medicine, 2nd edition by Cleveland Clinic | *Current Clinical Medicine, 2nd edition by Cleveland Clinic | ||
[[Category:GI]] | [[Category:GI]] | ||
Revision as of 07:59, 7 June 2015
Background
- Autoimmune Dz typically seen in young men
- Progressive inflammation and fibrosis of intra/extra hepatic bile ducts
- Most (80%) cases are associated with inflammatory bowel dz, typically ulcerative colitis, 10% of pts with ulcerative colitis have PSC
- Increased risk of colon CA in pts with Ulcerative colitis and PSC (more than UC alone) Increased risk of cholangiocarcinoma
- Prevalence is 1 to 6 per 100,000 in the U.S
Clinical Features
- Generally asymptomatic but can present with fatigue, abd pain, jaundice, cholangitis, puritis, weight loss, or fever
- Mean age at presentation: 30-40
Differential Diagnosis
Diagnosis
- Cholangiography
- Dx made by ERCP or MRCP, which demonstrates strictures or beading of the intrahepatic or extrahepatic bile ducts
- Liver biopsy typically shows pericholangitis and periductual fibrosis but is often not diagnostic in early dz
Work-Up
- Alkaline phosphatase is usually elevated with mild elevations in aminotransferases
- Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved in late stages of dz
- Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases
Treatment
- High dose Ursodeoxycholic acid (UDCA), 25-30mg/kg/day, may improve liver chemistries but does not slow dz progression and may actually hasten development of portal HTN
- Periodic dilation of strictures via ERCP or percutaneous route
- Liver Transplant should be offered to those with advanced liver dz or repeated bouts of cholangitis (Dz can recur after transplantation)
Disposition
- Annual Screening for colon cancer in pts with concomitant UC
See Also
References
- Current Clinical Medicine, 2nd edition by Cleveland Clinic