Primary sclerosing cholangitis

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Primary sclerosing cholangitis (pronunciation: /ˈpraɪmɛri sklɪˈroʊsɪŋ koʊlænˈdʒaɪtɪs/) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts which normally allow bile to drain from the gallbladder. Affected individuals may have no symptoms or may experience signs and symptoms of liver disease such as yellow discoloration of the skin and eyes, itching, and abdominal pain.

Etymology

The term "Primary sclerosing cholangitis" is derived from the Greek words "skleros," meaning hard, "angeion," meaning vessel, and "itis," meaning inflammation. It refers to the hardening and inflammation of the bile ducts.

Symptoms

The symptoms of primary sclerosing cholangitis include jaundice, pruritus, abdominal pain, and fatigue. In some cases, the disease may be asymptomatic and is discovered during routine blood tests.

Causes

The exact cause of primary sclerosing cholangitis is unknown, but it is believed to be related to an abnormal immune response. The disease is often associated with inflammatory bowel disease, particularly ulcerative colitis.

Diagnosis

Diagnosis of primary sclerosing cholangitis can be challenging, as the symptoms are often non-specific and can be attributed to other conditions. The diagnosis is usually confirmed through a combination of blood tests, imaging studies, and sometimes a liver biopsy.

Treatment

There is currently no cure for primary sclerosing cholangitis, but treatment can help manage symptoms and slow the progression of the disease. Treatment options include medications to manage itching and bile flow, as well as procedures to open blocked bile ducts. In severe cases, a liver transplant may be necessary.

Prognosis

The prognosis for individuals with primary sclerosing cholangitis varies. Some individuals may live with the disease for many years without experiencing serious symptoms, while others may develop serious complications such as cirrhosis, liver failure, or cholangiocarcinoma, a type of bile duct cancer.

See also

External links

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