It is believed that primary sclerosing cholangitis may be caused by autoimmune destruction of bile ducts. However, there are competing theories and the mechanism is not fully understood.
Histologically, fibrosis of bile ducts resembles the layers of an onion. This is known as concentric periductal fibrosis.
This is visible on radiographs of the intrahepatic and extrahepatic biliary tree. "Beading” is a manifestation of the strictures and dilations of the bile ducts that occur due to inflammation and fibrosis.
Itchiness, along with fatigue, are often presenting symptoms of primary sclerosing cholangitis. However, similar to other cholestatic causes of pruritus, this mechanism is poorly understood.
Chronic and extensive liver inflammation and fibrosis that is progressive and pervasive in primary sclerosing cholangitis lends itself to the development of hepatosplenomegaly.
As the disease process and liver dysfunction progress, bilirubin metabolism and excretion malfunctions and accumulates, resulting in the characteristic yellow discoloration of the skin called jaundice. Similarly, dark urine may also be present in these patients, manifesting kidney excretion of excess bilirubin.
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