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What are symptoms of primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive (worsening over time) disease of the bile ducts.
– This disease channelizes bile from the liver into the intestines.
– Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine, particularly fat.
– This liquid also helps to get rid of the body of toxins.
– Liver cells secrete the bile they make into small canals within the liver.
– The bile flows through the canals and into larger collecting canals (ducts).
– The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts.
– From the extrahepatic bile ducts, the bile flows into the intestine where the bile mixes with food.

In primary sclerosing cholangitis :
– The intrahepatic and extrahepatic bile ducts become inflamed,
– scarred and thickened (sclerotic),
– narrowed,
– finally obstructed.

Obstruction of the ducts can lead to:
– abdominal pain,
– itching,
– jaundice,
– infection in the bile ducts (cholangitis),
– liver scarring that leads to liver cirrhosis,
– liver failure.

Symptoms of primary sclerosing cholangitis:
– Most patients with early primary sclerosing cholangitis have no symptoms.
– The presence of primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of liver enzymes (particularly alkaline phosphatase levels).
– This is often recognized along with a routine physical examination.
Early symptoms of primary sclerosing cholangitis include
– Fatigue
– bodily itching (pruritus)

With the progress of the disease:
– Patients may develop jaundice (yellowing of skin and darkening of urine).
– Jaundice is due to the accumulation of bilirubin in the body.
– The bilirubin accumulates because it is not able to be eliminated in the bile.
– This is due to prolonged obstruction of the bile ducts.
– The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow.
– It may be due to accumulation of bile salts in the body.
– It is as a result of obstruction of the bile ducts.

As primary sclerosing cholangitis progresses, patients typically develop:
– Right upper abdominal pain,
– Fever,
– Fatigue,
– Pruritus
– Jaundice
– These patients also are at risk of developing primary sclerosing cholangitis complications.
– The patients with the autoimmune form of primary sclerosing cholangitis have more rapid symptoms of abdominal pain, jaundice and fever.

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