Primary Sclerosing Cholangitis (PSC)

Primary sclerosing cholangitis is a disease that affects primarily the bile ducts that exist inside and outside the liver. The ducts of the gallbladder and pancreas may be compromised too.

In consists on an inflammation of the bile ducts (cholangitis), which causes fibrosis (scarring and hardening), narrowing the compromised ducts. This prevents bile from being properly drained through the ducts, and instead it accumulates itself within the liver, damaging this organ’s cells.

When there is a high amount of accumulated bile, it gets into the bloodstream and damages other tissues of the body. With time, the liver develops cirrhosis and ceases to function properly.

Causes Of Primary Sclerosing Cholangitis

Although the exact causes of primary sclerosing cholangitis (PSC) remain unknown, most experts seem to think that this condition is in fact related to an anomaly of the immune system. It is suspected that, under certain conditions, the immune system stops recognizing the liver as a part of the body, and begins attacking it as if it were a foreign threat. If this is true, then primary sclerosing cholangitis would be an autoimmune disease.

Some people also think that there are genetic reasons that may cause a predisposition for the development of PSC, and that there are also genetic reasons that link primary sclerosing cholangitis to ulcerative colitis, which is usually present in people with PSC.

Symptoms Of Primary Sclerosing Cholangitis

As PSC is a slow-progression disease, symptoms may not appear on early stages. The only indication of the possible presence of primary sclerosing cholangitis may be laboratory findings, usually an increased level of alkaline phosphatase and other enzymes.

As the disease progresses, symptoms start showing up. These symptoms may be intermittent or persistent, and they may become worse with time. Symptoms of PSC may include fever, itching (due to the bile being “injected” into the blood). Additionally, infection of the bile ducts may develop, and often abdominal tenderness is often observed.

When PSC is advanced enough, there may be loss of appetite, fatigue, weight loss and yellowing of eyes and skin (jaundice). After cirrhosis appears, there may be abdominal and feet swelling.

Treatment Of Primary Sclerosing Cholangitis

PSC is, at least for now, not curable. However, there are many treatments for primary sclerosing cholangitis that are effective in relieving the symptoms and delaying the progression of the disease.

There are many drugs that allow a patient to slowly eliminate the excess of bile in the body, and others that effectively decrease its production. Additionally, the use of these drugs has proven to be helpful in stopping or delaying further damage to the liver, increasing life expectancy.

If an infection is present, it may be treated with antibiotics. Swollen feet and abdominal pain may be treated with anti-inflammatory medication. As cirrhosis causes liquid retention, diuretics are often prescribed.

In some cases surgery may be performed to widen the bile ducts and remove blockages, allowing bile to drain properly. When primary sclerosing cholangitis is in an advanced state, liver transplantation must be strongly considered.

Prevention Of Primary Sclerosing Cholangitis

As with most liver diseases, prevention of primary sclerosing cholangitis consists on avoiding alcohol consumption and following an adequate diet.

Bookmarks
Bookmark This |