Primary sclerosing cholangitis

Introduction

Primary sclerosing cholangitis is an inflammation of the final scar changes and blockage of bile ducts inside and outside the liver.

In primary sclerosing cholangitis scar changes narrows and eventually clog pipes causing cirrhosis. The cause is unknown but is likely to be associated with immune system abnormalities. The disease most often affects young males. It usually occurs in people with inflammatory bowel disease, particularly ulcerative colitis.

Symptoms and diagnosis

The disease usually begins gradually with the growing fatigue, itching and jaundice. There might be attacks of pain in upper abdomen and fever caused by inflammation of the bile ducts, but they are uncommon. The infected person may have increased the liver and spleen or symptoms of cirrhosis. A person can develop and the portal hypertension, ascites and liver failure, which can lead to death.

The diagnosis is usually made by endoscopic retrograde cholangiopancreatography (ERCP) or percutaneous cholangiography. In ERCP-in, after the bile ducts through the endoscope injected radiocontrast material, make the roentgenogram. In percutaneous cholangiography, radiographs are made after direct injection of radiocontrast agents bile ducts. To confirm the diagnosis may be needed Microscopic sample of liver tissue obtained hollow needle (liver biopsy).

Treatment and Prognosis

Some people have no symptoms even 10 years (after the disease is discovered by routine tests of liver function).Primary sclerosing cholangitis is usually progressively worsens.

Medications such as corticosteroids, azathioprine, penicillamine, and methotrexate have been shown to be very effective, and can cause serious side effects. Value ursodiola is unclear. Primary sclerosing cholangitis may require liver transplantation, which is the only known cure of this disease or death.

Repeated infections of bile ducts (bacterial cholangitis) is a complication of the disease and requires treatment with antibiotics. Narrow lines can be extended endoscopic or surgical procedure. In 10% - 15% of people with primary sclerosing cholangitis developed biliary tract cancer (cholangio). The tumor grows slowly and requires the use of a treatment of endoscopic procedures in order to put stents in bile ducts that the patient keep lines open. Sometimes it is necessary surgical treatment.

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