Primary Sclerosing Cholangitis

Related Subjects: | Chronic liver disease | Cirrhosis | Alkaline phosphatase (ALP) | Liver Function Tests | Ascites Assessment and Management | Budd-Chiari syndrome | Autoimmune Hepatitis | Primary Biliary Cirrhosis | Primary Sclerosing Cholangitis | Wilson disease | Hereditary Haemochromatosis | Alpha-1 Antitrypsin (AAT) deficiency | Non alcoholic steatohepatitis (NASH) | Spontaneous Bacterial Peritonitis | Alcoholism and Alcoholic Liver Disease

In later stages of the disease liver transplantation is often an option. Survival after this operation is good, although in some patients PSC can recur in the new liver.

About

• Progressive fibrosis and inflammation and destruction of the intrahepatic and extrahepatic bile ducts
• Cholangiocarcinoma develops in 10 to 15% of patients. Ulcerative colitis seen in 75%

Aetiology

• There is an association with inflammatory bowel disease in over 50% of cases
• Can be classified as primary and secondary due to another disease

Clinical

• Progressive jaundice possibly in a patient with IBD
• Malaise, itch, weight loss
• Episodes of cholangitis - fever, jaundice, RUQ pain

Investigations

• Isolated raised Alkaline phosphatase at first
• Mildly elevated AST and ALT
• Raised Bilirubin (normal initially) and cholestasis
• +ve ANA and anti-smooth muscle
• +Ve Anti Mitochondrial suggests Primary biliary cirrhosis
• Positive pANCA is positive in 2/3rd but non-specific
• Raised gamma globulins
• USS - exclude gallstones and other causes of cholestasis
• ERCP may be used but MRCP is non-invasive and the imaging of choice and shows strictures with intervening normal areas. "Beaded appearance"
• Histology - classical onion skin of bile ductules

Staging

• Stage I : Inflammation in portal tracts
• Stage II : Hepatitis and portal fibrosis
• Stage III: Bridging fibrosis
• Stage IV: Biliary cirrhosis and nodules

Management

• No treatment slows progression. Nutrition and fat-soluble vitamins
• Stenting for strictures and balloon dilatation
• Immunosuppressives ineffective
• Pruritis: Treat with cholestyramine for the itch.
• Ursodeoxycholic acid improves Bilirubin, ALP and Albumin but not survival
• Liver transplant with Choledochojejunostomy as the disease can otherwise recur
• Transplanted patients need an annual colonoscopy for colonic cancer
• Median survival is 12 years

Complications

• Cholangiocarcinoma
• Colonic carcinoma with Ulcerative colitis-associated PSC

References

• Cholangitis: Diagnosis, Treatment and Prognosis