Primary Sclerosing Cholangitis Causes, Symptoms, Diagnosis, Treatment, Home Remedies

Primary Sclerosing Cholangitis Causes, Symptoms, Diagnosis, Treatment, Home Remedies

What is primary sclerosing cholangitis?

Primary sclerosing cholangitis (PSC) is an uncommon condition that affects the bile ducts and liver. It is called:

Primary Sclerosing Cholangitis Causes, Symptoms, Diagnosis, Treatment, Home Remedies

  • Primary - because the cause is not known. (That is, it is not 'secondary' to any known cause such as alcohol or poisons.)
  • Sclerosing - because it causes scarring and thickening (sclerosis) of the bile ducts.
  • Cholangitis - which means inflammation of the bile ducts.
In this condition, the bile ducts both inside and outside the liver become inflamed and scarred. The scarring causes narrowing of these bile ducts which results in bile building up in the liver. The bile can then damage the liver cells. Eventually, the scar tissue can spread throughout the liver, causing cirrhosis and liver failure

Cirrhosis is a serious condition where normal liver tissue is replaced by scar tissue (fibrosis). It tends to progress slowly and often does not cause symptoms in its early stages. However, as the function of the liver gradually becomes worse, serious problems can develop.

What causes primary sclerosing cholangitis?

It's not clear what causes primary sclerosing cholangitis. It's believed that the condition may be caused by an immune system reaction to an infection or toxin in people with a predisposition to develop the disease.

Primary sclerosing cholangitis occurs more frequently in people with certain other medical conditions, particularly inflammatory bowel disease. It's not clear how these conditions are linked and whether they share similar causes.

Who gets primary sclerosing cholangitis?

Most people with PSC are adults but the disease also occurs in children. The average age at diagnosis is 40. PSC is more common in men than women. Having family members with PSC may increase a person's risk for developing PSC.

What are the symptoms of primary sclerosing cholangitis?

Signs and symptoms of primary sclerosing cholangitis include:

  • Abdominal pain
  • Chills
  • Diarrhea
  • Fatigue
  • Fever
  • Itchiness
  • Weight loss
  • Yellowing of your eyes and skin (jaundice)
Primary sclerosing cholangitis may not cause any symptoms in its early stages. In some cases, the only indication of this disorder may be abnormal blood tests suggesting that your liver isn't functioning well.

When to see a doctor 

Make an appointment with your doctor if you have any persistent signs and symptoms of primary sclerosing cholangitis.

Risk factors

Factors that may increase the risk of primary sclerosing cholangitis include:

  • Your age. Primary sclerosing cholangitis can occur at any age, but it's most commonly diagnosed in people ages 25 to 45.
  • Your sex. Primary sclerosing cholangitis occurs more often in men than it does in women.
  • Inflammatory bowel disease. The great majority of people with primary sclerosing cholangitis also have inflammatory bowel disease, which includes ulcerative colitis and Crohn's disease. Still, primary sclerosing cholangitis is rare among people with inflammatory bowel disease, and most won't develop primary sclerosing cholangitis. It's not clear why these diseases occur together. If you've been diagnosed with primary sclerosing cholangitis, your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms.

What are the complications of primary sclerosing cholangitis?

Various complications can occur in some people with PSC. These include:

  • Deficiencies of some vitamins, usually vitamins A, D, E and K. These are the vitamins which are fat-soluble (rather than the other vitamins which are water-soluble). This means they dissolve in fat. Bile helps the fat to be broken down and these vitamins to be absorbed.
  • Infection of the blocked bile ducts. This is called infective cholangitis. This may cause chills, fever and upper tummy (abdominal) tenderness.
  • Cirrhosis (where normal liver tissue is replaced by scar tissue (fibrosis).
  • Liver failure.
  • Bile duct cancer. This eventually affects around 1 in 10 people who have PSC.

How is primary sclerosing cholangitis diagnosed?

Many people with PSC have no, or only vague, symptoms for quite some time in the early stages of the disease. Therefore, the diagnosis is often made when you have tests for an unrelated condition or routine tests if you have inflammatory bowel disease.

Tests usually include:

  • Blood tests called liver function tests. These measure the activity of chemicals (enzymes) and other substances made in the liver. This gives a general guide as to whether the liver is inflamed, and how well it is working. See separate leaflet called Liver Function Tests for more detail.
  • Other blood tests may be performed to rule out (exclude) other causes of liver conditions such as viral hepatitis.
  • An ultrasound scan of the liver may be performed.
  • A cholangiogram is a test which produces a picture of the bile ducts. This is often done using an MRI scan.
  • Taking a small sample (biopsy) of the liver. This may be carried out to look at the sample under the microscope. It can show inflammation and the extent of any cirrhosis (where normal liver tissue is replaced by scar tissue (fibrosis) in the liver). The liver biopsy can also assess how early or advanced the disease is. See separate leaflet called Liver Biopsy for more detail.

What is the treatment and Drug for primary sclerosing cholangitis?

At present there is no specific treatment which either cures or slows the progression of PSC. Treatments aim to improve symptoms and also to manage any complications which may arise.

Treatments for primary sclerosing cholangitis focus on reducing signs and symptoms of the disease as it progresses. Primary sclerosing cholangitis progresses slowly, but it usually ends in liver failure and the need for a liver transplant. Many medications have been studied in people with primary sclerosing cholangitis, but so far none has been proved to slow or reverse the liver damage associated with this disease.

Treatment for itching 

Medications that may help control itching include:

  • Antihistamines, such as diphenhydramine (Benadryl, others)
  • Bile-acid-binding drugs, such as cholestyramine (Locholest, Questran, others)

Treatment for infections 

Bacterial infections can occur frequently in people with primary sclerosing cholangitis. Antibiotics may be prescribed to treat an infection. If you've had repeated infections, your doctor may recommend antibiotics before procedures that are likely to cause infection, such as endoscopic procedures or surgery.

Treatment for malnutrition 

Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins. Even though you may eat a healthy diet, you may find that you can't get all the nutrients you need. Your doctor may recommend vitamin supplements that you take as tablets or that you receive as an infusion through a vein in your arm.

Treatment for bile duct blockages 

Blockages that occur in your bile ducts can be treated with:

  • Balloon dilation and stent placement. These procedures can open blockages in the larger bile ducts. Balloon dilation is a procedure in which your doctor runs a slender tube with an inflatable balloon at its tip (balloon catheter) through an endoscope and into a blocked bile duct. Once the balloon catheter is in place, the balloon is inflated. Small plastic tubes called stents may be placed in bile ducts to keep them open.

  • Bile duct surgery. In certain situations, blockages in bile ducts may need to be removed surgically. After removing a blockage, the surgeon connects the remaining portions of bile duct so that bile can still flow through the duct.

Liver transplant 

A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. Liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it's possible for primary sclerosing cholangitis to recur after a liver transplant.

Lifestyle and home remedies for Primary Sclerosing Cholangitis

  • Don't drink alcohol.
  • Quit smoking. If you don't smoke, don't start.
  • Get vaccinated against hepatitis A and B.
  • Follow directions on over-the-counter medications.
  • Maintain a healthy weight.
  • Use care with chemicals at home and at work.
  • Talk to your doctor about any herbs or supplements you're taking, since some can be harmful to your liver.

Clinical Trials

Clinical trials are research studies that test new ways to detect, prevent or treat disease. Mayo Clinic conducts more than 3,000 clinical trials and research studies each year and often coordinates national clinical trials with other medical centers.

Learn more about clinical trials and whether Mayo Clinic may be conducting a clinical trial related to your condition or procedure.

Points to Remember

  • Primary sclerosing cholangitis (PSC) inflames, scars, and blocks bile ducts inside and outside the liver.
  • When bile ducts become blocked, bile builds up in the liver and damages liver cells.
  • PSC can lead to vitamin deficiencies, infections, bile duct cancer, cirrhosis, liver failure, and the need for a liver transplant.
  • The cause of PSC is not known.
  • Many people with PSC also have ulcerative colitis, an inflammatory bowel disease.
  • Treatment includes medications to treat symptoms and complications of PSC.