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Primary Sclerosing Cholangitis - Symptoms
Medical expert of the article
Last reviewed: 06.07.2025
Men are affected twice as often as women. Primary sclerosing cholangitis usually develops at the age of 25-45 years, but it is possible even in children aged 2 years (average age 5 years), usually in combination with chronic nonspecific ulcerative colitis.
Most often, the onset of the disease is asymptomatic; the first manifestation, especially in screening examination of patients with nonspecific ulcerative colitis, is an increase in the activity of serum alkaline phosphatase. However, primary sclerosing cholangitis can be detected cholangiographically even with normal alkaline phosphatase activity. The disease can also initially manifest itself as an increase in the activity of serum transaminases. It is on the basis of this sign that it can be accidentally detected in donors when donating blood. Even with an asymptomatic course, the disease can progress with the development of liver cirrhosis and portal hypertension, usually presinusoidal, without signs of cholangitis or cholestasis. Such patients can be treated for many years for "cryptogenic" cirrhosis.
Typically, primary sclerosing cholangitis begins with weight loss, fatigue, pruritus, right upper quadrant pain, and transient jaundice. The presence of symptoms indicates that the disease is advanced. Fever is uncommon unless ascending cholangitis develops as a result of biliary surgery or endoscopic examination. However, the disease sometimes begins with fever, chills, right upper quadrant pain, pruritus, and jaundice, resembling acute bacterial cholangitis. Blood cultures are rarely positive, and antibiotics are ineffective.
Always, even in the absence of symptoms of bowel disease, nonspecific ulcerative colitis (and in rare cases Crohn's disease) should be excluded by performing a rectoscopy and biopsy of the rectal mucosa. Colitis is usually chronic, diffuse, mild to moderate. The activity of cholangitis is inversely proportional to the activity of colitis. Remissions are usually long-term. Primary sclerosing cholangitis may be detected earlier or later than colitis. The presence of nonspecific ulcerative colitis does not affect the course of the disease.
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