Cholangitis

What is cholangitis?

Cholangitis means inflammation in your bile ducts, the tubes that carry bile from your liver to other organs in your digestive system.

Your liver makes bile and sends it to your gallbladder for storage, which sends it to your small intestine to help with digestion. Bile is made up of waste products from your blood and bile acids that help break down fats. Bile helps with digestion in your small intestine and helps carry waste products out of your body through your large intestine.

If your bile ducts are inflamed, they’re irritated, swollen and possibly infected. Infection is the most common cause of cholangitis, but not the only cause. Infection causes acute cholangitis (ascending cholangitis), which is a temporary response from your immune system. Chronic diseases can cause chronic cholangitis, the kind that doesn’t go away.

What happens when you have cholangitis?

Inflammation in your bile ducts can cause several serious complications:

• Swelling can obstruct the flow of bile, causing bile to back up into your organs.
• When bile backs up into your organs, it causes inflammation in those organs.
• Bile that doesn’t flow is a breeding ground for bacterial infections if you don’t have one already.
• An infection in your bile ducts can spread to your bloodstream, which would be life-threatening.
• Chronic inflammation causes scarring. Scar tissue in your bile ducts can cause them to corrode.
• Narrowed and corroded bile ducts will permanently obstruct the flow of bile through the ducts.

Is cholangitis life-threatening?

It can be. Different types are dangerous in different ways. The most immediate threat comes from a bacterial infection in your bile ducts. If the bacteria spreads to your liver, it could enter your bloodstream through the blood vessels in your liver. This would cause a systemic infection (septicemia), which could lead to sepsis, a whole-body shock reaction that shuts down your vital organs.

Sometimes acute inflammation is caused by a blockage in your bile ducts, like a gallstone. The blockage causes bile flow to stall, and this can lead to secondary infection. If you have chronic cholangitis, it can slowly destroy your bile ducts over many years. This affects your liver. When chronic inflammation causes scarring in your liver, it slowly stops functioning. Liver failure is life-threatening.

What is the difference between cholangitis vs. cholecystitis?

Cholangitis is inflammation in your bile ducts, and cholecystitis is inflammation in your gallbladder. They look and sound similar because they both begin with the prefix “chole-,” which means bile. (“Gall-” also means bile, so gallbladder actually means bile bladder.) The suffix “-itis” means inflammation. Bile flows through your bile ducts to and from your gallbladder, and inflammation in one can affect the other.

What are the causes of cholangitis?

Acute causes include:

• Infection from bacteria, a virus or parasite.
• Blockages such as gallstones, strictures and tumors.
• Cholestasis, stalled bile flow.

In most cases (about 90%), these three factors work together to cause cholangitis. A blockage, usually a gallstone in the common bile duct (choledocholithiasis), causes bile flow to stall, and infection follows. Bile is supposed to flow one way into your gallbladder, and from there, into your small intestine. But internal pressure from stalled bile can cause bacteria to backwash into the bile ducts from these organs.

However, a blockage in your bile ducts can cause cholestasis and cholangitis without a bacterial infection. And you can have cholestasis without a blockage. Certain diseases, medications or long-term IV feeding can cause it. You can also get an infection without a blockage. It’s less likely, but it’s possible it could come through your blood or from a medical instrument introduced during a test or procedure.

Autoimmune diseases that cause chronic cholangitis include:

• Primary sclerosing cholangitis.
• Primary biliary cholangitis.
• IgG4-associated cholangitis.

These conditions cause your immune system to mistakenly attack cells in your body as foreign invaders. The constant immune response causes constant inflammation in your bile ducts. It’s usually not as severe as acute cholangitis, and you may not become aware of it for years. But over time, it causes scarring in your bile ducts. This in turn may lead to obstruction, cholestasis and acute cholangitis.

What does cholangitis feel like?

You might not notice chronic cholangitis for years. Many people aren’t diagnosed until chronic cholangitis has become advanced enough to affect their livers, causing symptoms of liver disease. Acute cholangitis will make itself known, though. You’ll almost certainly feel pain in your upper right abdomen, which might radiate to your right shoulder blade or back. It may be sharp or dull, and it may come and go.

If your bile ducts are obstructed, you may experience what’s known as “biliary colic.” This abdominal pain comes in episodes, typically after a heavy meal. It’s triggered by your gallbladder contracting to push bile out to your small intestine to help with digestion. Pushing bile against the obstruction causes pressure and pain, often with nausea. It builds quickly to a peak and then gradually wanes after that.

What are the symptoms of cholangitis?

The most common symptoms of acute cholangitis are known as Charcot’s Triad. They include:

• Upper right quadrant abdominal pain. The upper right is where your biliary system is located.
• Fever. Fever typically accompanies inflammation when your immune system is activated.
• Jaundice. The yellow tint to your skin and eyes comes from bile leaking into your bloodstream, a sign that your bile ducts are backed up.

Depending on how severe your condition is, you might have other symptoms related to backed-up bile leaking into your bloodstream, like:

• Pruritus. Bile acids in your bloodstream can cause skin itching.
• Hepatic encephalopathy. Toxins from bile in your bloodstream can cause mild cognitive impairment, such as confusion and disorientation.

If you have an infection that has entered your bloodstream, you may have symptoms of shock, such as:

• Fast heart rate (tachycardia).
• Low blood pressure (hypotension).

If you have Charcot’s Triad of symptoms plus signs of confusion and signs of shock, it’s known as Reynolds’ pentad. This combination signifies to your healthcare provider that your condition is critical.

If you have chronic cholangitis, you may not have symptoms until your condition is advanced. Symptoms may include all of the above, as well as:

• Fatigue.
• Swollen abdomen.
• Swollen legs and feet.
• Fat deposits under your skin.
• Large, visible veins across your abdomen.
• Spider veins.
• Dark-colored pee and light-colored poop.
• Diarrhea.

How is cholangitis diagnosed?

Your healthcare provider will examine you for typical symptoms, then take blood tests and imaging tests of your biliary system. Blood tests can identify an infection if you have one and tell your provider how much bile is circulating in your bloodstream. They can also measure liver enzymes and proteins to check how your liver is doing. Imaging tests can show whether your bile ducts are obstructed, and where.

Blood tests may include:

• Complete blood count (CBC).
• Liver function tests.

Imaging tests may include:

• Magnetic resonance cholangiopancreatography (MRCP).
• Abdominal ultrasound.

What is the treatment for cholangitis?

The primary treatment for both acute and chronic cholangitis is endoscopic biliary drainage. Once your healthcare provider has identified the source of the problem, they’ll work to relieve pressure and restore flow through your bile ducts. They can usually do this during an endoscopy, a procedure that involves passing a long, thin tube with a camera attached down your throat and into your bile ducts.

Providers can drain excess bile through the tube to relieve pressure. If necessary, they can also remove blockages and place stents to prop open the bile ducts, using tiny tools passed through the tube. If you have an endoscopic procedure to diagnose cholangitis, your provider can treat it at the same time. If you have chronic cholangitis, you might need repeat procedures to relieve symptoms from time to time.

Additional treatment for chronic cholangitis may include:

• Antihistamines to treat itching.
• Vitamin supplements to treat vitamin deficiencies.
• Ursodeoxycholic acid (UDCA) to slow the progression of primary biliary cholangitis.

Additional treatment for acute cholangitis may include:

• IV fluids.
• Pain relief.
• Antibiotics.

What is the prognosis for people with cholangitis?

Whether you have acute or chronic cholangitis, it’s a scary position to be in. Both can be life-threatening, either sooner or later. But acute cholangitis is usually brief. With timely treatment, most people recover completely. Without treatment, acute cholangitis has a high mortality rate.

If you have chronic cholangitis, you may live without complications for years. Medications are often effective in treating symptoms. You may need periodic biliary drainage procedures. In some people, liver disease can progress to liver failure. If this happens, you might eventually need a liver transplant.

How do I take care of myself while living with chronic cholangitis?

If you have chronic biliary disease, it’s important to take care of your liver in the ways that you can. A healthy diet and lifestyle can minimize the damage and help extend the life of your liver. Try to avoid toxins that stress your liver, including alcohol, drugs, smoke and other environmental chemicals. Try to minimize saturated fats and sugar in your diet. Get some exercise and reduce stress as much as possible.

A note from Cleveland Clinic

Bile plays an important role in your body, but it only works when it can flow freely. When it can’t, it leaks into your bloodstream and backs up into your organs, causing toxicity and inflammation. That’s why healthy bile ducts are so important. If you have biliary pain and suspect it’s cholangitis, seek treatment as soon as possible. Whether cholangitis is chronic or acute, earlier treatment has the best results.