Primary sclerosing cholangitis is a condition where bile ducts are inflamed and scarred. The patient may die due to complications like liver failure, bleeding or cancer.
Sclerosing Cholangitis is a condition where the bile ducts are inflamed, leading to scarring and narrowing of the ducts with resultant complications.
Liver and Bile Ducts
The liver consists of two parts, the left and right lobe. It produces a juice called bile that helps in digestion of fat from food. Bile travels through a number of small channels in the liver. These channels are called intra-hepatic bile ducts. Smaller ducts join to form bigger ducts and they eventually emerge from the liver as two main channels, the left and right hepatic ducts. These ducts join to form the common hepatic duct. The common hepatic duct in turn joins the cystic duct coming from the gall bladder to form the common bile duct. The gall bladder normally stores the excessive bile produced by the liver. The common bile duct finally opens into the first part of the intestine.
Sclerosing cholangitis causes inflammation of bile ducts, either within the liver or outside the liver. Inflammation leads to scarring and narrowing of some portions of the bile ducts. Thus, the flow of bile from the liver to the intestine may be obstructed. The bile accumulates in the liver causing liver damage.
In primary sclerosing cholangitis (PSC), the cause of the disease is not known. It often affects men in their forties, especially those also suffering from inflammatory bowel disease like ulcerative colitis. In addition, bile duct stones, surgery, parasites or drugs are responsible for some cases of sclerosing cholangitis.
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The patient may not suffer from any symptoms or he may experience fever, fatigue, weight loss, itching, jaundice and pain in the abdomen. He may also suffer from complications like vitamin deficiency, bone disease, bile duct infection, liver failure and cancer of the bile ducts.
The patient is diagnosed using blood tests like liver function tests and imaging studies like endoscopic retrograde cholangiography and magnetic resonance cholangiography. Liver biopsy helps in the diagnosis of the condition.
Surgery is usually required to treat sclerosing cholangitis. A procedure often used is dilatation of the bile ducts using endoscopic retrograde cholangiography. Sometimes, a stent is inserted in addition in the narrow part to keep the duct open. Liver transplantation is necessary in patients with liver failure. A drug, ursodeoxycholic acid may be useful in the early stages.