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Primary sclerosing cholangitis (PSC) is a liver disease that causes inflammation and scarring in the bile ducts, making them shrink over time. (Bile ducts are small tubes that bile flows through from within the liver to the gallbladder and intestines). This inflammation results from immune cells attacking the bile ducts. PSC is called “primary” because the cause is unknown, “sclerosing” because of the scarring, and “cholangitis” because the inflammation occurs in the bile ducts.

Bile is a yellow-green fluid produced by your liver to aid digestion. When bile ducts are damaged by inflammation and scarring (cholangitis), bile and other substances collect in the liver, which in turn damages liver cells.

PSC may sometimes be confined to the bile ducts within the liver, but most often it also attacks the large bile ducts outside the liver. The disease affects both sexes, but it’s more common in men. It occurs in people mostly between the ages of 30 and 50.

Symptoms

The progression of PSC is unpredictable and not well understood. A person can have the disease for many years before symptoms show. The most common symptoms include:

• Chronic fatigue. This can range from mild to disabling.
• Itching. Known medically as pruritus, itching can range from mild to intense. It can be relentless, disrupting sleep and quality of life. This itching usually attacks the whole body and may be the result of the liver’s inability to process bile.
• Jaundice. This refers to yellowing of the skin and eyes.
• Episodes of fever, chills, and abdominal pain. These may be signs of an infection within the bile ducts. Any patient with PSC who has fever and chills should go to the nearest hospital emergency room immediately.

In its advanced stages, PSC may lead to extensive scarring in the bile ducts and eventually, liver failure.

As liver damage progresses in PSC, other symptoms may appear. These can affect parts of your body outside your liver. These symptoms are the same as for advanced liver disease from any cause.

Diagnosis

Because people with PSC may have no symptoms for many years, PSC is often diagnosed when patients show abnormal results from standard blood tests. Additional diagnostic tests such as MRI (magnetic resonance imaging) or ERCP (endoscopic retrograde cholangiopancreatography) may be necessary to confirm PSC.

ERCP combines the use of X-ray and an endoscope, which is a long, flexible tube. Through this form of endoscopy, your doctor can see inside your stomach and duodenum and inject dyes into your bile ducts. This creates a high-quality X-ray that can show strictures (narrowing) caused by inflammation in your bile ducts.

Treatment

There is no specific treatment for slowing the advance of PSC. Unfortunately, PSC is relentless over time. It leads to liver failure and the need for liver transplant with almost all patients.

However, doctors can treat PSC symptoms with varying success using antibiotics, vitamins, and medications.

To help relieve itching (pruritus), a common symptom, your doctor may prescribe various medications such as cholestyramine, antihistamines, or other drugs such as naltrexone or rifampicin. For patients with extreme itching, doctors will occasionally prescribe ultraviolet light therapy.

Sometimes, doctors will use ERCP to dilate a stricture or insert a stent in the bile ducts. This may alleviate jaundice and other symptoms over the short term.

Causes

The causes of primary biliary cholangitis (PBC) remain unknown. But current evidence suggests that PBC occurs in people with a genetic predisposition—in other words, there may be a hereditary factor. However, there may also be other factors that combine with genetics to trigger the disease.

Medical scientists believe that PBC is an autoimmune disease. This means that your immune system, which protects against infections and cancer, mistakes cells in the bile ducts as being abnormal and attacks them. (The bile ducts are small tubes that bile flows through from within the liver to the gallbladder and intestines.) Some factors—such as an infection or some form of toxic exposure from the environment—may trigger your immune system into making this mistake.

PBC usually develops and progresses slowly, which means that liver damage typically gets worse over a long period of time. Medication can slow this progression, especially if you get treated early. It’s important to note that PBC is not caused by alcohol consumption.

PBC used to be known as primary biliary cirrhosis.

Symptoms

The most common symptoms of PBC are fatigue and itching in any part of the body.

Many people have chronic fatigue. This can range from mild to disabling.

Itching, which is referred to medically as pruritus, can also range from mild to intense. It can be unrelenting to the point of creating stress for some patients. It often occurs on palms or soles of the feet and may be the result of the liver’s inability to process bile.

Additional PBC symptoms might include:

• gradual darkening of the skin
• small yellow raised areas under the skin (usually at the inside corners of the eyes)

Other symptoms not directly related to PBC are also often reported by people with PBC:

• arthritis
• dry membranes (nose, eyes, mouth, vagina)
• fingers and/or toes that change colour in the cold (Raynaud’s disease)
• thyroid problems

If liver damage gets worse, other symptoms may appear. These can affect parts of your body outside your liver. These symptoms are the same as for advanced liver disease from any cause.

Many people with PBC never develop any symptoms related to the disease.

Diagnosis

PBC is usually diagnosed with the following blood tests:

• Antimitochondrial Antibody (AMA). An antibody is a protein made by your body’s immune system to attack an “invader.” AMA is an important indicator of PBC.
• Alkaline Phosphatase (ALP or Alk-Phos). Another sign of PBC is increased levels of ALP (also commonly called alk-phos). Alk-phos is an enzyme released into the blood by damaged bile ducts.
• Alanine Aminotransferase (ALT) and Aspartate Aminotransferase (AST). Increased levels of these enzymes may not be as common in PBC as increased alk-phos. They measure inflammation relating mainly to liver cell damage or destruction rather than cell damage in the bile ducts. (Note: ALT is also known as alanine transaminase. AST is also known as aspartate transaminase.)
• Immunoglobulin (IgM). Increased levels of IgM can also be an indicator of PBC.

Other tests might include:

• Abdominal ultrasound. This is done to check the condition of the bile ducts and ensure there’s no other cause of decreased bile flow out of the liver.
• Liver biopsy. Doctors use this test to view the condition of the liver at the cellular level.

Treatment

PBC is a chronic liver disease: a lasting condition that can be controlled in most people but not cured. The prognosis for people with PBC has improved over the last two decades due to earlier diagnosis and improved treatment. Early access to treatment can delay the progression of the disease. Treatment improves outcomes for people with PBC, and sometimes, it can even improve outcomes so that they are similar to people without the disease.

Medications

A drug called ursodeoxycholic acid (also called UDCA or URSO) mimics a bile acid your body produces naturally. URSO can improve liver function and slow the onset of fibrosis (scar tissue) in the liver. In turn, this can delay or eliminate the possibility of liver failure and the need for a liver transplant. 80 to 90% of people with PBC on URSO manage to avoid the need for a liver transplant after 10 years. Patients usually take this drug indefinitely. Fortunately, it’s well tolerated and has minimal side effects.

Researchers are working on new medicines to treat PBC, typically for use with URSO. Obeticholic acid (OCA), a manufactured bile acid that helps reduce liver inflammation and cholestasis (restricted bile flow), is available in Canada.

To help relieve itching (pruritus), a common symptom, your doctor may prescribe various medications such as cholestyramine, antihistamines, or other drugs such as naltrexone or rifampicin. For people with extreme itching, doctors will occasionally prescribe ultraviolet light therapy.

Other Treatments

Your doctor will also discuss other treatments for you to consider. These can include calcium and vitamin D to preserve your bone health. It may also involve other fat-soluble vitamin supplements and treatments for managing symptoms such as dry eyes, mouth, or vagina.

The best way to describe autoimmune hepatitis is to break down the term “autoimmune.” Autoimmune refers to the body’s own immune system attacking another part of the body. In this case, the immune system attacks the liver and causes liver inflammation.

Autoimmune hepatitis is not contagious. Also, it’s not considered an inherited disease. But a tendency to autoimmune diseases may run in some families.

Causes

We don’t yet know exactly why or how the body’s immune system attacks its own liver. But we do know it happens because of a mistake made by certain types of white blood cells, the cells that fight infections. It appears that white blood cells misread liver cells as harmful foreign substances and attack them. This causes liver inflammation. With time, this ongoing inflammation might lead to fibrosis (scarring of the liver) and eventually cirrhosis (severe scarring of the liver).

Several other things can cause identical patterns of liver damage. These include viruses such as hepatitis B and hepatitis C, certain types of drugs, and an overload of certain metals in the liver such as copper and iron. There are also other causes that are very rare.

Symptoms

In many people, there are no symptoms at all. They feel perfectly healthy. Other people may experience:

• aches or pains in the muscles or joints
• decreased appetite
• drowsiness
• fatigue
• jaundice (yellowing of the eyes and skin)

Diagnosis

Autoimmune hepatitis may be detected with a series of blood tests.

The initial clue from blood tests is high levels of ALT and AST, which are two enzymes made by the liver. However, ALT and AST levels go up in many liver diseases or conditions, so the next step to diagnose autoimmune hepatitis is to measure specific antibodies directed against parts of different cells. These are called autoantibodies.

Your doctor will also examine you and may find signs such as a large liver and jaundice.

Finally, a liver biopsy is usually necessary to confirm the diagnosis, determine the extent of liver damage, and ensure there aren’t co-existing liver conditions.

Treatment

Since we don’t know exactly what causes autoimmune hepatitis, we don’t yet have a medical cure. But we do have effective treatments.

If the disease is mild or inactive, you might not need any type of treatment. When medications are needed, corticosteroids are the first treatment of choice. Corticosteroids are different from anabolic steroids, which are used to build muscle mass. Corticosteroids such as prednisone are potent anti-inflammatory drugs. They reduce inflammation throughout the body, including the liver. There are side effects, however, so your doctor will try to use the smallest amount possible to control the disease.

Your doctor may also prescribe other anti-inflammatory drugs such as azathioprine, methotrexate, or mycophenolate mofetil. All these drugs have side effects your doctor will explain in detail. These can also help to limit the dose of corticosteroids you require.

After drugs have reduced the initial inflammation caused by autoimmune hepatitis, about 90% of people need ongoing treatment to stay in remission. This treatment is usually azathioprine. About 10% don’t need ongoing treatment. They remain in remission with no symptoms and no active inflammation in the liver for many years. But they do require monitoring with simple blood tests.

Since the intensity of the disease goes up and down, some people suffer from relapses—even those who have been receiving ongoing treatment. Relapses are usually treated by a temporary course of corticosteroids.

Note: This section was adapted (with permission) from content on the website of the Canadian Liver Foundation.

Cannabis

Cannabis (marijuana) is a drug that people may use it for medical or non-medical reasons. ​​​​​​​​​​​​​​​​​​​When you use cannabis, you may be putting your health at risk.

Unfortunately there have not been enough studies to show whether cannabis can be damaging to your liver. The Canadian Association on Gastroenterology believes that cannabis may increase scarring of your liver (hepatic fibrosis) and thus does not recommend its use in patients with cirrhosis.

Cannabis can cause side effects like anxiety, nausea, dizziness and changes to heart rate and blood pressure. Although it is legal in many regions, it’s still important you discuss use with your healthcare provider.

Smoking

Smoking is dangerous to everyone’s health. People with liver disease are more vulnerable to infection and to poor health, so smoking or exposure to passive smoking is not recommended.

To learn more about quitting smoking see the useful links below: