Cholangitis is inflammation of the bile ducts of the liver. Sclerosis is inflammation that leads to the extensive formation of fibrous and scar tissue.
In primary sclerosing cholangitis (PSC), the bile ducts inside and outside the liver have become inflamed and scarred.
PSC is a type of cholangitis thought to be caused by an autoimmune reaction. As the scarring increases, the bile ducts become blocked or narrowed, leading to an accumulation of liver-damaging bile (a liquid that assists in the breakdown of fat in food).
The blockage of bile transport to the gut results in jaundice, and eventually causes liver failure.
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What Causes PSC?
The exact cause of PSC is not known. Various theories – including the role of chronic or recurrent bacterial infection, viruses, genetic predisposition, ischemic damage (relating to lack of blood) to the biliary tree, and immune system problems – are currently being researched.
PSC is associated with ulcerative colitis (a type of inflammatory bowel disease that frequently causes abdominal pain and bloody diarrhea), and it is believed that these diseases share a common cause.
However, while ulcerative colitis is in the colon, PSC affects the biliary tree. Unfortunately, PSC persists in spite of the surgical removal of the colon, if needed for management of IBD.
Because PSC progresses slowly, the disease can be present for many years before symptoms appear.
The predominant symptoms are:
- Bile duct infection (can cause chills and fever)
- Intense itching
- Malabsorption (especially of fat) leading to decreased levels of the fat-soluble vitamins, A, D, E, and K.
- Severe jaundice or icterus (causing yellowing of the eyes and/or skin)
- Signs of cirrhosis
- Steatorrhea (a build-up of fat in stool, and loose, greasy, foul bowel movements)
PSC is more common in men than women; approximately 70% of patients with PSC are men diagnosed at around 40 years of age.
Onset normally begins between the age of 30 and 60, but it can also occur during childhood.
PSC is diagnosed through what is known as cholangiography, which involves injecting dye into the bile ducts, and taking an X-ray.
Cholangiography can be performed as an endoscopic procedure (using a small, flexible tube with a light and a lens to look into the esophagus, stomach, duodenum and pass dye through the bottom of the bile duct), through radiology (with MRI) or even surgery.
Other tests include a full blood count; measuring liver enzymes, and electrolyte levels; and examining renal function.
Stool tests may be required to measure the amount of fat in feces, especially when malabsorption symptoms are pronounced.
PSC Prognosis and Treatment
- Medication to relieve itching (antipruritics)
- Antibiotics to treat infections when they occur
- Bile acid sequestrants (cholesterol-lowering medication)
- Vitamin supplements as people with PSC are often deficient in vitamins A, D, and K.
- In some cases, surgery or an ERCP (endoscopic procedure) to open major blockages in the common bile duct is also necessary.
Between 75 and 90% of patients with PSC have inflammatory bowel disease (IBD). However, only around 40% of patients with IBD have or develop PSC.
PSC may lead to cirrhosis of the liver with portal hypertension. A liver transplant may be needed if the liver begins to fail.
Primary sclerosing cholangitis (PSC) is now the fourth leading indication for liver transplantation in adults.
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