Primary sclerosing cholangitis: Difference between revisions
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== Background | ==Background== | ||
*Autoimmune | *Autoimmune disease typically seen in young men | ||
*Progressive inflammation and fibrosis of intra/extra hepatic bile ducts | *Progressive inflammation and fibrosis of intra/extra hepatic bile ducts | ||
*Most (80%) cases are associated with inflammatory bowel | *Most (80%) cases are associated with [[inflammatory bowel disease]], typically [[ulcerative colitis]], 10% of patients with ulcerative colitis have PSC | ||
*Increased risk of colon | *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 | *Prevalence is 1 to 6 per 100,000 in the U.S | ||
== Clinical Features | ==Clinical Features== | ||
*Generally asymptomatic but can present with fatigue, [[abdominal pain]], [[jaundice]], cholangitis, | *Generally asymptomatic but can present with fatigue, [[abdominal pain]], [[jaundice]], [[cholangitis]], [[pruritus]], weight loss, or [[fever]] | ||
*Mean age at presentation: 30-40 | *Mean age at presentation: 30-40 | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Autoimmune hepatitis | |||
*Primary biliary cirrhosis | |||
*IgG4 related disease | |||
== | ==Evaluation== | ||
*Alkaline phosphatase is usually elevated with mild elevations in aminotransferases | *[[LFTs]] | ||
*Bilirubin is typically normal, except when common hepatic duct or common bile duct is involved in late stages of disease | **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 | *Perinuclear antineutrophil cytoplasmic antibody (pANCA) positive in 2/3rds of cases | ||
*Cholangiography | *Cholangiography | ||
*Diagnosis made by ERCP or MRCP, which demonstrates strictures or beading of the intrahepatic or extrahepatic bile ducts | *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 | *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 | *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 | **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 | *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) | *Liver Transplant should be offered to those with advanced liver disease or repeated bouts of cholangitis (disease can recur after transplantation) | ||
== Disposition | ==Disposition== | ||
*Annual Screening for colon cancer in patients with concomitant [[ulcerative colitis]] | *Annual Screening for colon cancer in patients with concomitant [[ulcerative colitis]] | ||
== See Also | ==See Also== | ||
==References== | ==References== | ||
Latest revision as of 17:53, 18 August 2022
Background
- Autoimmune disease typically seen in young men
- Progressive inflammation and fibrosis of intra/extra hepatic bile ducts
- Most (80%) cases are associated with inflammatory bowel disease, typically 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, abdominal pain, jaundice, cholangitis, pruritus, weight loss, or fever
- Mean age at presentation: 30-40
Differential Diagnosis
- Autoimmune hepatitis
- Primary biliary cirrhosis
- IgG4 related disease
Evaluation
- 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 ulcerative colitis
See Also
References
- Current Clinical Medicine, 2nd edition by Cleveland Clinic