What is primary sclerosing cholangitis (PSC)

~ by Jo Jordan

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Cholangitis is inflammation of the bile ducts of the liver. Sclerosing 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, 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.

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 – a systemic ailment affecting many areas of the body), and it is believed that these diseases share a common cause.

However, while the cause of ulcerative colitis is in the colon, the cause of PSC is suspected to be located outside the colon because PSC persists in spite of the surgical removal of the colon.¹

PSC symptoms

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)
Fatigue
Intense itching
Malabsorption (especially of fat) leading to decreased levels of the fat-soluble vitamins, A, D, E, and K.
Severe jaundice (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 seventy percent of patients with PSC are men diagnosed at around 40 years of age.² Onset – normally beginning between the age of 30 and 60 – can also occur during childhood.

Diagnosing PSC

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, colon, or rectum), through radiology or surgery, or with magnetic resonance imaging (MRI) scans.

Other tests include a full blood count; measuring liver enzyme, bilirubin, and electrolyte levels; and examining renal function. Stool tests may be required to measure the amount of fat in feces, especially when malabosorption symptoms are pronounced.

PSC prognosis and treatment

Treatment includes medication to relieve itching (antipruritics), antibiotics to treat infections, bile acid sequestrants (cholesterol-lowering medication), and vitamin supplements as people with PSC are often deficient in vitamins A, D, and K. In some cases, surgery to open major blockages in the common bile duct is also necessary.

Between seventy-five and ninety percent of patients with PSC have inflammatory bowel disease (IBD). However, only around four percent of patients with IBD have or develop PSC.³

PSC may lead to cirrhosis of the liver with portal hypertension. Liver transplantation may be indicated if the liver begins to fail.

Primary sclerosing cholangitis (PSC) is now the fourth leading indication for liver transplantation in adults.⁴

For more information on the liver, please review the liver cleansing overview article, or search for related articles by using the search box.

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Notes:
1. 1. Wikipedia.org, "Primary sclerosing cholangitis," http://en.wikipedia.org/wiki/Primary_sclerosing_cholangitis (accessed November 7, 2007).
2. Vikas Khurana, MD, eMedicine.com, "Primary Sclerosing Cholangitis,"http://www.emedicine.com/med/topic3556.htm (accessed November 7, 2007).
3. Ibid.
4. Ibid.