Primary sclerosing cholangitis (PSC) is a progressive disease characterized by inflammatory strictures involving the intrahepatic and extrahepatic biliary tree.
-It eventually results in secondary biliary cirrhosis
-Men are more commonly affected than women (70:30 ratio), with a mean age at onset near 40 years.
A vast majority of patients (prevalence rates range from 70% to 90%) have underling inflammatory bowel disease, especially ulcerative colitis.
-Smoking is associated with a decreased risk of primary sclerosing cholangitis in patients who also have inflammatory bowel disease.
-Coffee consumption is also associated with a decreased risk of primary sclerosing cholangitis.
Symptoms & Signs
Fatigue, right upper quadrant abdominal pain, pruritus, jaundice, and acute cholangitis
Labs: Disproportionate elevation of serum alkaline phosphatase; hypergammaglobulinemia, increased serum IgM levels, p-ANCA
Imaging: MRCP is the diagnostic study of choice with “beads on a string” apperance
Liver biopsy: The most characteristic histologic finding is ‘onion skin pattern due to fibrous obliteration of small bile ducts with concentric replacement by connective tissue
-There is no specific proven treatment for PSC
-Medical management is largely supportive
-Pruritis: Cholestyramine, naltrexone, rifampin, doxepin
-surveillance colonoscopy with biopsies should be carried out at 1–2-year intervals.
-screen for osteopenia at 2–3-year intervals after an initial evaluation.
-Liver transplantation is best choice for patients with advanced PSC
-Complications: Fat-soluble vitamin deficiencies (A,D,E and K), Metabolic bone disease, cholangitis, cholelithiasis, gallbladder cancer, cholangiocarcinoma, liver cancer, colon cancer
-Patients with PSC have an 8–15% lifetime risk of developing cholangiocarcinoma.
-With liver transplantation, 5-year survival is up to 85%