Primary sclerosing cholangitis produces fever, chills, and abdominal pain. Inability of the body to properly manage bile may turn the skin yellow temporarily. The majority of people with the disorder also have inflammatory bowel disease. Experts believe there is a strong genetic aspect to who develops the condition, but this disposition does not guarantee someone will get it.
Primary biliary cirrhosis has symptoms similar to primary sclerosing cholangitis. Symptoms may develop slowly over five to 20 years. In the most severe cases, a liver transplant is required. Diagnosis is most likely after age 40. Approximately 90% of all patients with the disorder are women, and prevalence is higher in those of European descent.
Many cases of both primary biliary cirrhosis and primary sclerosing cholangitis are diagnosed before symptoms are severe. This is because about 70% of primary biliary cirrhosis patients experience serious fatigue that leads them to see a doctor. Fatigue is also the most common symptom in its sister disease and tends to be noticed first.
Neither condition should be confused with acute cholangitis. Acute cholangitis is characterized by bile duct damage caused by temporary blockage of the ducts. The blockage inhibits bile flow, which leads to infection. Acute cholangitis requires emergency hospitalization.