A Guide to Cholangitis

Medically Reviewed By Youssef (Joe) Soliman, MD
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Cholangitis is inflammation of the biliary system. The biliary system consists of your gallbladder, liver, pancreas, and bile ducts. This system produces, stores, and moves bile around your body. There are three main types of cholangitis: primary, acute (or secondary), and immune. Acute cholangitis is the most common form, but it is still relatively rare. It affects fewer than 200,000 people per year in the United States.

Read on to learn more about the causes, symptoms, and treatments related to cholangitis.

Sex and gender terms

Sex and gender exist on spectrums. For the purposes of this article, we use the terms “male” and “female” to refer to a person’s sex assigned at birth.

What are the types of cholangitis?

A man laying down with his hands on his stomach and head
Shikhar Bhattarai/Stocksy United

The three main types of cholangitis are primary, acute, and immune.

Primary cholangitis

Doctors typically split primary cholangitis into two subtypes: primary biliary and primary sclerosing. Although these two forms can be very similar, the main difference is that primary biliary cholangitis only affects the bile ducts inside the liver. In contrast, primary sclerosing cholangitis affects the bile ducts inside and outside the liver.

Primary biliary cholangitis mostly affects females during middle age. Primary sclerosing cholangitis primarily affects males of European origin, with a higher prevalence in the third and seventh decades of life.

Acute cholangitis

Acute cholangitis is the most common form of cholangitis, and it typically results from a bacterial infection in your bile ducts. It frequently occurs in people ages 50–60 years, with males and females equally affected.

Immune cholangitis

Immune cholangitis, also known as IgG4-related sclerosing cholangitis, occurs when IgG4-positive lymphocytes infiltrate the biliary system. It occurs predominantly in males in their 50s and 60s.

What are the causes of cholangitis?

The different types of cholangitis have different causes.

Experts do not know exactly what causes primary cholangitis. Primary sclerosing cholangitis may result from genetic factors, immune system disorders, bacterial changes in the digestive tract, or trauma to the bile ducts. Genetics may play a role in the development of primary biliary cholangitis, with symptoms resulting from environmental factors such as infections or chemical exposures.

Acute cholangitis typically results from a bacterial infection of the bile ducts. An obstruction of biliary flow — usually in the form of gallstones — causes the infection. The obstruction increases biliary pressure, meaning that bile backs up and allows bacteria to grow.

Although experts do not know exactly why it happens, immune cholangitis occurs when your immune system starts to consider your biliary system a threat. The immune system sends out lymphocytes, which are a type of white blood cell, and this causes inflammation.

What are the symptoms of cholangitis?

The classic symptoms of cholangitis are a high fever persisting for longer than 24 hours, abdominal pain, and jaundice. This presentation is called Charcot’s triad.

Other symptoms of cholangitis may include:

How do doctors diagnose cholangitis?

The standard for diagnosing cholangitis is to use the Tokyo Guidelines. Under these guidelines, doctors evaluate:

  • Clinical features: Doctors look for fever or chills, jaundice, and abdominal pain. They will also ask you about any previous disorders affecting the biliary system.
  • Laboratory features: Doctors will run tests to look for certain inflammatory markers, such as elevated leukocyte counts or C-reactive protein positivity.
  • Imaging findings: The results of X-rays, endoscopy, ultrasounds, CT scans, and MRI scans can help doctors determine whether or not there are any structural anomalies, such as blockages.

Under these criteria, finding two or more clinical features constitutes a suspected diagnosis. A definitive diagnosis is either the presence of Charcot’s triad or two clinical features in addition to both laboratory and imaging findings.

What are the treatments for cholangitis?

The goal of treatment for cholangitis is to manage inflammation, infection, and biliary obstruction.

Primary sclerosing cholangitis treatment may involve endoscopic retrograde cholangiopancreatography. During this procedure, a healthcare professional will open up narrowed or blocked bile ducts and keep them open using stents.

Doctors typically treat primary biliary cholangitis with medications. These medications can slow liver damage progression and improve inflammation.

The mainstay of treatment for acute cholangitis is antibiotics to fight off pathogens in the biliary system. In severe cases, doctors may recommend IV antibiotics and hospitalization. For people who are improving with antibiotics, biliary decompression or drainage may be necessary before hospital discharge.

Immune cholangitis usually requires corticosteroids. You may also need to have stents placed in your biliary system to relieve pressure.

What are some potential complications of cholangitis?

Cholangitis complications can range in severity from mild disease to failure of multiple organs. Specifically, these complications can include:

What are the risk factors for cholangitis?

Several factors can increase your risk of developing cholangitis, including:

  • an increased triglyceride intake
  • a sedentary lifestyle
  • rapid weight loss
  • heavy alcohol consumption
  • certain autoimmune conditions, such as inflammatory bowel disease
  • HIV

People who are more prone to gallstones may be more likely to develop cholangitis. Gallstones are more common in Native American and Hispanic people, less common in white people, and much less common in Asian American and African American people.

What is the outlook for people with cholangitis?

The outlook for people with cholangitis depends on the type they have and how early doctors can make a diagnosis and begin treatment.

Life expectancy has improved to over 10 years for 80% of people with primary sclerosing cholangitis who receive a liver transplant. On the other hand, people with primary biliary cholangitis may have a poor outlook if they already have jaundice when they receive their diagnosis.

With prompt treatment, the outlook for people with mild cases of acute cholangitis is generally good. Around 80–90% respond well to treatment and have a positive outlook. People who present with early signs of multiple organ failure need immediate biliary drainage. After this procedure, the overall mortality rate decreases to less than 10%.

People with immune cholangitis who respond well to steroid therapy typically have a favorable outlook. Older adults and those with certain comorbidities — such as cirrhosis, malignancy, or liver abscess — may have a worse outlook.

Summary

Cholangitis is a condition wherein the biliary system becomes inflamed. Infections, genetic factors, immune system disorders, and trauma can all play a role in the development of cholangitis.

Doctors typically diagnose cholangitis with a clinical examination, laboratory tests, and imaging tests. Treatment may include taking medications, undergoing procedures to drain bile, or getting surgery to open up blocked or narrowed bile ducts.

Early diagnosis and treatment are essential for improving your outlook with cholangitis. Talk with your doctor if you are experiencing symptoms consistent with cholangitis.

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Medical Reviewer: Youssef (Joe) Soliman, MD
Last Review Date: 2022 Jun 15
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