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What is Primary Sclerosing Cholangitis and what are its causes?

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.

How common is primary sclerosing cholangitis?
– Primary sclerosing cholangitis is a rare disease.
– An estimated prevalence in the United States of 6 per 100,000 people.
– It is more common in men then in women; approximately 70% of primary sclerosing cholangitis patients are men.
– The mean age at diagnosis of primary sclerosing cholangitis is around 40 years of age.
– There is a strong association between primary sclerosing cholangitis and chronic ulcerative colitis.
– Primary sclerosing cholangitis can also occur alone or in association.
– This usually occurs with Crohn’s disease, a disease of the intestines that is related to ulcerative colitis.

What causes primary sclerosing cholangitis?
– The cause of primary sclerosing cholangitis is not known.
– A small subset (approximately 10%) of primary sclerosing cholangitis patients has a rapidly progressive form of the disease.
– This includes early onset of abdominal pain, and itching.
– This responds dramatically to treatment with corticosteroids.
– Since corticosteroids (such as prednisone) are medications for treating immune diseases primary sclerosing cholangitis patients are believed to have an immune disorder that causes their primary sclerosing cholangitis.

Immune diseases include:
– Ulcerative colitis
– Crohn’s disease
– Systemic lupus erythematosus

Primary Sclerosing Cholangitis at a Glance
– Primary sclerosing cholangitis is a chronic, progressive disease of the liver.
– The cause of primary sclerosing cholangitis is not known.
– Though an immune cause is suspected in at least a minority of patients.
– Primary sclerosing cholangitis has a strong association with ulcerative colitis and cancer of the bile ducts.
– The primary symptoms of primary sclerosing cholangitis are due to obstruction of the bile ducts and also cirrhosis of the liver.
– Primary sclerosing cholangitis is diagnosed on the basis of abnormal blood tests and radiologic imaging of the bile ducts.
-Primary sclerosing cholangitis is treated with medications, endoscopy, and liver transplantation.

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