Primary sclerosing cholangitis

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at May 28, 2016
StartDiseasesPrimary sclerosing cholangitis

A slowly progressing chronic disease damaging the bile ducts, primary sclerosing cholangitis (PSC) causes bile to accumulate in the liver. This digestive liquid made by the liver gradually damages the major organ's own cells, leading to cirrhosis or fibrosis. As the resulting cirrhosis worsens and scar tissue from PSC increases, the liver becomes less able to function and bile ducts may become blocked, leading to infection and cancer.

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Definition & Facts

Primary sclerosing cholangitis is a bile duct disease. The bile ducts carry bile, a digestive fluid, to the small intestine from the liver. In a patient with primary sclerosing cholangitis, inflammation occurs in the bile ducts, leading to scarring of the bile ducts with these scars eventually hardening, narrowing the ducts, and seriously damaging the liver.

Primary sclerosing cholangitis is a slowly progressing disease that can cause repeated infections, tumors in the liver or bile ducts, and even liver failure. The disease's only known cure is a liver transplant. Care of patients with primary sclerosing cholangitis is focused upon monitoring of liver function, management of symptoms and temporary reopening of blocked bile ducts through procedures designed for that purpose.

The disease may be present for years before patients notice signs or symptoms. Symptoms may stabilize, progress slowly or intermittently appear. Liver failure often occurs about ten to 15 years following diagnosis of this disease, and often warrants liver transplant or leads to bile duct cancer.

Symptoms & Complaints

When symptoms are present early in the disease, patients experience fatigue and itching. This itching may be severe and otherwise unexplained, causing the patient to scratch without alleviation of the irritation. After the disease has progressed for several years, patients may experience the following:

Because of bile's role in digesting fat, primary sclerosing cholangitis prevents the body from absorbing crucial vitamins that are fat-soluble. Due to the inability to absorb important vitamins such as vitamin D, weakened bones and weight loss may result.

Causes

Primary sclerosing cholangitis' causes are unclear. The inflammation that occurs in the bile ducts may be caused by an immune system reaction to toxins or an infection. It may also be genetically rooted. There has been research that shows that having a family history of the disease may increase one's risk. Many patients diagnosed with with this condition also have inflammatory bowel disease, such as ulcerative colitis or Crohn's disease. Risk factors for primary sclerosing cholangitis include:

  • Age, with most diagnosed patients being between 30 and 50 years
  • Gender, as most patients are men
  • Prior or concurrent diagnosis of inflammatory bowel disease
  • Geography, with northern Europeans and Americans being more at risk than Asians and Southern Europeans

Diagnosis & Tests

Primary sclerosing cholangitis may be present years before being diagnosed, and it is often discovered by doctors through routine blood tests or X-rays for other conditions before symptoms are present. To diagnose primary sclerosing cholangitis, the following tests and procedures may be used:

  • Liver function blood tests. For this test, liver function is checked through enzyme levels.
  • Bile duct X-rays. An endoscopic retrograde cholangiopancreatography (ERCP) may be ordered with or without an MRI. A flexible tube is inserted down the throat to inject dye into the small intestines at the bile ducts. This dye makes the region's details more visible through imaging.
  • Liver tissue sampling and testing. A liver biopsy removes a small sample of liver tissue for close examination in a laboratory. The doctor inserts a needle into the liver through the skin and removes a tissue sample which is then tested to determine how much liver damage has occurred. This test is only conducted when X-rays, blood tests and other tests have not led to a certain diagnosis of primary sclerosing cholangitis.

Treatment & Therapy

Patients diagnosed with primary sclerosing cholangitis are treated with the goal of management of complications and monitoring of damage to the liver. Although many medications have been utilized in trials for patients with PSC, none have yet been discovered to slow or reverse liver damage attributed to the disease. 

Symptomatic itching may be treated through antihistamines or medications that bind to bile acids. Another option for itching if a bile acid-binding drug is not well tolerated is prescription of rifampicin, an antibacterial medication. This drug is believed to block the brain's recognition of itching.

Vitamin absorption can be a problem for patients with PSC, so doctors often recommend vitamin supplementation through oral supplements or infusion. Calcium and vitamin D supplements are particularly important.

Infections caused by backed up bile may be prevented through repeated courses of antibiotics or prolonged dosing of these drugs. When bile duct blockages occur, these blockages may be treated through the following means:

  • Balloon dilation in larger bile ducts using an endoscope
  • Placement of a stent to hold ducts open, also using an endoscope

Liver transplant is the only known cure for primary sclerosing cholangitis. The diseased liver is removed and replaced with a healthy one from a donor. This only occurs when liver failure has occurred or complications of the disease have become severe. Uncommonly, primary sclerosing cholangitis can recur in patients after a liver transplant.

Patients diagnosed with PSC are advised to ensure their best health through the following measures:

Prevention & Prophylaxis

Because the cause of the disease is not known, it is difficult to prevent. There is some belief that viral infections or bacterial infections may be a contributing factor toward primary sclerosing cholangitis. Genetics may also be a factor, allowing individuals with relatives suffering from the disease to seek early diagnosis. Healthy diet and regular exercise contribute to one's overall health and may help stave off disease or render a person more equipped to fight disease.