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 

Diagnosis 

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 

Treatment 

-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

Prognosis 

-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%