View source for Primary sclerosing cholangitis

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==Background==

*Autoimmune disease typically seen in young men <ref>Lazaridis KN, LaRusso NF. Primary Sclerosing Cholangitis. N Engl J Med. 2016;375(12):1161-1170. PMID 27653566</ref>
*Progressive inflammation and fibrosis of intra/extra hepatic bile ducts 
*Most (80%) cases are associated with [[Special:MyLanguage/inflammatory bowel disease|inflammatory bowel disease]], typically [[Special:MyLanguage/ulcerative colitis|ulcerative colitis]], 10% of patients with ulcerative colitis have PSC 
*Increased risk of colon cancer in patients 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, [[Special:MyLanguage/abdominal pain|abdominal pain]], [[Special:MyLanguage/jaundice|jaundice]], [[Special:MyLanguage/cholangitis|cholangitis]], [[Special:MyLanguage/pruritus|pruritus]], weight loss, or [[Special:MyLanguage/fever|fever]]
*Mean age at presentation: 30-40


==Differential Diagnosis==

*Autoimmune hepatitis
*Primary biliary cirrhosis
*IgG4 related disease


==Evaluation==

*[[Special:MyLanguage/LFTs|LFTs]]
**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 disease
*Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases
*Cholangiography 
*Diagnosis 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 disease
*Consider serum IgG4 levels


==Management==

*High dose Ursodeoxycholic acid (UDCA), 25-30mg/kg/day
**May improve liver chemistries but does not slow disease progression and may actually hasten development of portal hypertension 
*Periodic dilation of strictures via ERCP or percutaneous route 
*Liver Transplant should be offered to those with advanced liver disease or repeated bouts of cholangitis (disease can recur after transplantation)


==Disposition==

*Annual Screening for colon cancer in patients with concomitant [[Special:MyLanguage/ulcerative colitis|ulcerative colitis]]


==See Also==


==References==

*Current Clinical Medicine, 2nd edition by Cleveland Clinic 

[[Category:GI]]
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