Primary sclerosing cholangitis
(Redirected from Primary Sclerosing Cholangitis)
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