Primary sclerosing cholangitis (PSC) is an inflammatory disease of the bile ducts, leading to a host of symptoms and complications and, in later stages, liver failure. The cause of PSC is unknown, though research has linked several genetic and environmental risk factors. PSC is degenerative, resulting in progressive deterioration of the liver and bile ducts. Early detection, complication treatment and management, and long-term care are crucial to slowing the disease's progress and relieving symptoms.
The bile ducts are part of the biliary system, a tract joining the liver to the duodenum, the first section of the small intestine. The liver produces bile, which travels to the duodenum along this tract and aids in the digestion of fats. Bile is also stored in the gallbladder and released during digestion.
In primary sclerosing cholangitis, inflammation leads to scarring and narrowing of the bile ducts. In repairing the damage caused by inflammation, fibrous connective tissue or scar tissue forms on the walls of the ducts. The build-up of this scar tissue can eventually block the ducts and cause deterioration.
The cause of primary sclerosing cholangitis is unknown, though genetic and environmental risk factors appear to play a significant role in its development. PSC is hereditary, and people with specific genes associated with immunity are more likely to be diagnosed with the disease. Those genetically predisposed to PSC may develop the disease following exposure to an environmental trigger or injury to or infection of the bile ducts.
Doctors often diagnose PSC alongside one of several common co-occurring conditions. These include connective tissue disorders and autoimmune or immunodeficiency diseases. The condition most commonly associated with PSC is inflammatory bowel disease, particularly ulcerative colitis. The chronic condition causes inflammation and deterioration of the walls of the bowel or large intestine.
Primary sclerosing cholangitis progresses slowly, presenting with minor symptoms that worsen over time. The first symptoms are usually fatigue and relentless itching, called pruritis. Abdominal pain, fever, weight loss, and jaundice — yellowing of the skin and eyes — often present in the later stages. Complications often arise as the disease progresses, resulting in acute symptoms.
Chronic scarring and narrowing of the bile ducts may lead to complications. A person with primary sclerosing cholangitis may develop gallstones and bile duct stones or recurring bacterial inflammation of the ducts. Impaired utilization of fat and vitamins A, D, E, and K may develop, resulting in decreased bone density, bleeding, and bruising. PSC also places a person at a higher risk of developing cancer of the bile ducts. The most common and critical complication of PSC is chronic liver disease or cirrhosis.
Cirrhosis occurs as a result of extensive scarring of the liver. Symptoms include
Advanced cirrhosis may result in liver failure.
A doctor will diagnose primary sclerosing cholangitis with a liver function test, a blood test that measures inflammation and protein-production in the liver. Ultrasonography may also identify blockages in the bile ducts. Ultrasonography uses sound waves to produce images of the biliary system. Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) can confirm a diagnosis.
Symptoms of the disease and its complications — itching, bacterial inflammation, and portal hypertension — are treated with prescription medication. Doctors may also suggest vitamins, supplements, and dietary restrictions. Surgery to place a stent to dilate the bile ducts is a temporary method of managing the disease. A liver transplant is typically the only treatment option for extensive liver damage or advanced cirrhosis.
There is no cure for PSC other than a liver transplant, but patient and doctor can often manage the advancement and symptoms of the disease. Regular medical care, liver function tests, and prescribed medicines, vitamins, and treatment regimens can significantly slow progression. This is particularly true if PSC is diagnosed early, so those with a family history of the disease need to inform their doctors of this possible predisposition.
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