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Primary sclerosing cholangitis: symptoms
Last reviewed: 23.04.2024
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Men get sick 2 times more often than women. Primary sclerosing cholangitis usually develops at the age of 25-45 years, but it is possible even in children 2 years old (mean age 5 years), usually in combination with chronic ulcerative colitis.
Most often the onset of the disease is asymptomatic; the first manifestation, especially in the screening of patients with ulcerative colitis, is an increase in serum alkaline phosphatase activity. Nevertheless, primary sclerosing cholangitis can be detected cholangiographically even with normal alkaline phosphatase activity. The disease can also initially be manifested by an increase in the activity of serum transaminases. It is on the basis of this sign that it can be accidentally detected in blood donors. Even with asymptomatic disease, the disease can progress with the development of cirrhosis and portal hypertension, usually a presynusoid, with no signs of cholangitis or cholestasis. Such patients can be treated for many years from "cryptogenic" cirrhosis.
Usually at the beginning of primary sclerosing cholangitis, there is a decrease in body weight, fatigue, itching, pain in the upper right quadrant of the abdomen, transient jaundice. The presence of symptoms indicates a far-gone process. The fever is uncharacteristic, if as a result of operations on the biliary tract or endoscopic examination, ascending cholangitis does not develop. Nevertheless, sometimes the disease begins with fever, chills, pain in the right upper quadrant of the abdomen, itching and jaundice, reminiscent of acute bacterial cholangitis. Sowing blood rarely gives positive results, antibiotics are ineffective.
Always, even in the absence of symptoms of bowel disease, ulcerative colitis (and, in rare cases, Crohn's disease), should be excluded by performing a sigmoidoscopy and biopsy of the rectal mucosa. Colitis usually is chronic, diffuse, mild or moderate. The activity of cholangitis is inversely proportional to the activity of colitis. Remissions are usually prolonged. Primary sclerosing cholangitis can be detected sooner or later than colitis. The presence of nonspecific ulcerative colitis does not affect the course of the disease.
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