Cholangitis overview: What you should know

Last updated Aug. 16, 2024, by Marisa Wexler, MS
Fact-checked by Marta Figueiredo, PhD

Cholangitis is a liver condition characterized by inflammation in the bile ducts. It most commonly develops due to a bacterial infection in these ducts, but also may be caused by blockages of the bile ducts, cancers, parasites, or autoimmune disorders.

People with cholangitis most commonly experience abdominal pain and/or fever, and have a yellowish color to their eyes and skin. However, a wide range of other cholangitis symptoms may occur. Without prompt treatment, and depending on its cause, the disease can lead to life-threatening complications such as whole-body infection and liver failure.

What is cholangitis?

The term cholangitis refers to inflammation of the bile duct system, a series of tubes that essentially serve as a highway to move bile — the fluid made by the liver that helps break down certain fats and proteins during digestion — through the body. These ducts carry bile between the liver and the intestines where digestion occurs, as well as to and from the gallbladder, an organ located under the liver where bile is stored until it’s needed.

Bile duct inflammation can lead to duct damage, and ultimately to liver damage.

Cholangitis is not a common medical condition, but it does affect men and women equally. The disease shows different prevalence across ethnicities: Estimates suggest it’s more prevalent among Native American and Hispanic people than those of European descent in the U.S., and even less prevalent among Asian and African American individuals. It is more common among those between 50 and 60 years old.

Acute vs. chronic cholangitis

Cholangitis can come on suddenly, usually due to an infection or blockage in the bile ducts. This is called acute or ascending cholangitis. When bile duct inflammation develops slowly and is more long-lasting, it is called chronic cholangitis. Chronic forms of the disease usually develop as complications of lifelong health conditions, such as autoimmune diseases.

Primary vs. secondary cholangitis

When there isn’t any other clear cause of bile duct inflammation, cholangitis is classified as primary. In contrast, secondary cholangitis refers to cases in which such inflammation develops as a result of other health problems, including infections, injury, or cancer.

Causes

Cholangitis causes differ mainly between acute and chronic disease.

Causes of acute cholangitis

The most common cause of acute cholangitis is a bacterial infection — most frequently by the bacteria Escherichia coli, Klebsiella, Enterobacter, Pseudomonas, or Citrobacter. These biliary system infections typically develop along with reduced or stalled bile flow, which is most frequently caused by gallstones, or hardened bile deposits that can form in the gallbladder. Gallstones can get stuck in bile ducts, obstructing flow.

Other cholangitis causes may include:

strictures, when the bile ducts become narrowed or pinched off
certain tumors in the biliary ducts, liver, and pancreas
parasites, including roundworm and tapeworm.

Risk factors for cholangitis include:

a previous history of gallstones
increased intake of triglycerides, a type of fatty molecule
a non-active or sedentary lifestyle
obesity
rapid weight loss
infection with human immunodeficiency virus (HIV), or acquired immune deficiency syndrome (AIDS)
recent medical procedures involving the bile duct or nearby body parts
travel to countries where there may be exposure to worms or parasites.

Causes of chronic cholangitis

Chronic cholangitis usually is caused by an autoimmune disease — disorders in which the body’s immune system accidentally starts attacking healthy cells in or around the bile ducts.

Having such a disease can lead to inflammation and, in the long-term, scarring, or sclerosis. That, in turn, may lead to bile duct narrowing, bile flow stalling, and a return of bile to the liver, where it causes further damage.

Autoimmune diseases that can cause chronic cholangitis include:

primary biliary cholangitis, or PBC, formerly known as primary biliary cirrhosis
primary sclerosing cholangitis, or PSC
IgG4-associated cholangitis.

PBC, which occurs more often in women, affects the bile ducts within the liver, while PSC, occurring predominantly in men, can affect the ducts inside or outside the organ. IgG4-associated cholangitis, in contrast, is a major manifestation of IgG4-related disease, an autoimmune disease that causes inflammation in several organs.

Secondary sclerosing cholangitis is another form of chronic cholangitis, which can be caused by surgical trauma to the bile ducts, longstanding biliary obstruction, or infectious or toxic cholangitis, among other factors. One example is sclerosing cholangitis associated with HIV, in which infection-related strictures in the biliary tract result in biliary obstruction and inflammation.

Symptoms

Common cholangitis symptoms include:

pain in the upper right part of the abdomen where the bile duct system is located
fever and/or chills
yellowing of the skin and eyes, known as jaundice
fatigue, lethargy, and/or malaise
Itching, called pruritus
nausea and vomiting
dark urine and/or pale stools
confusion or disorientation.

Cholangitis affects every person differently, therefore patients may not experience all of these possible symptoms. Further, symptom severity can vary from mild to life-threatening. The combination of abdominal pain, fever, and jaundice — sometimes called Charcot’s triad — is present in nearly all cases of acute cholangitis.

Symptoms also may vary according to cholangitis types. While acute forms usually cause obvious symptoms right away, signs of chronic cholangitis may not be noticeable until the disease progresses to cause damage to the liver. As chronic disease progresses, patients may also experience weight loss and enlargement of the spleen — common to both primary PBC and PSC.

Given that cholangitis can be a serious liver disease, patients experiencing these symptoms are advised to seek medical attention without delay.

Diagnosis

If cholangitis is suspected, a range of medical tests may be employed to confirm the diagnosis and rule out other potential causes for these symptoms. Blood tests often are used to look for signs of inflammation, infection, and liver damage.

Imaging technologies used to visualize the bile ducts and surrounding structures are the main method for reaching a definitive cholangitis diagnosis. These technologies can help identify blockages and restricted bile flow, as well as signs of inflammation and tissue damage. The most common imaging methods used to identify cholangitis include:

noninvasive abdominal ultrasound
noninvasive magnetic resonance cholangiopancreatography
minimally invasive endoscopic retrograde cholangiography, or ERCP
minimally invasive endoscopic ultrasound.

In endoscopic procedures, a clinician typically uses a long flexible tube, equipped with a tiny camera, to see inside the body.

Treatment

Treatment strategies for cholangitis depend on the underlying cause of the disease.

Antibiotics

Because most cases of cholangitis are caused by bacterial infections in the bile duct, antibiotics — medications that can kill bacteria — are a mainstay of cholangitis treatment. Typically, patients are given intravenous, or into-the-vein, antibiotics that can get into the liver and bile ducts in high concentrations.

Surgery

In both acute and chronic cholangitis, medical procedures to help drain excess fluid from the bile ducts and surrounding tissues are commonly used. The gold standard is ERCP, a minimally-invasive procedure that combines X-ray imaging with endoscopy, or the insertion of a thin, flexible tube-like instrument into the body through a natural opening, such as through the mouth.

As ERCP also can help diagnose cholangitis, the procedure may simultaneously be used to diagnose and also treat cholangitis in patients showing signs highly indicative of the bile duct disease.

Biliary decompression or drainage in cholangitis also can be achieved through another minimally invasive procedure called endoscopic ultrasound-guided drainage. More invasive approaches, including percutaneous transhepatic cholangiography and open surgery, may be needed in more severe cases.

In people with chronic types of cholangitis, repeated surgical procedures to drain built-up fluid may be needed from time to time.

Stenting

A biliary stent or bile duct stent is a thin, hollow, but firm tube that can be placed in a bile duct to help prop the duct open. Stents may be placed when there is an obstruction like a stricture that is preventing normal bile flow; by keeping the ducts more open, stents can help normalize the flow of bile. In most cases, stents can be placed with the ERCP tube-like instrument.

Liver transplant

Liver transplant, a surgical procedure in which a patient is given a new liver from a donor, is considered the only definitively curative treatment for cholangitis. This procedure may be considered in patients with severe liver disease or those with substantial symptoms that aren’t responsive to other treatments.

Other supportive measures

Other measures sometimes are used for cholangitis treatment.

Pain medications and intravenous administration of fluids may be used in some cases to manage symptoms of acute cholangitis.

People with chronic cholangitis may also be given antihistamines, or medications that help reduce allergic reactions, to treat itching. Mineral and vitamin supplements may be used for potential dietary deficiencies due to problems in absorbing nutrients.

In people with PBC, first-line therapy comprises ursodeoxycholic acid, a nontoxic bile acid that helps slow liver damage, while second-line therapies include Ocaliva (obeticholic acid), Iqirvo (elafibranor), and Livdelzi (seladelpar).

In addition, chronic cholangitis patients often are advised to maintain a healthy, well-balanced diet, and avoid eating foods that have a high risk of carrying infectious bacteria or viruses. Such foods include raw or undercooked shellfish, fish, meat, and unpasteurized milk. It’s often recommended that patients also avoid using drugs like alcohol and tobacco that can put strain on the liver.

Complications

When left untreated, acute cholangitis can cause fatal complications such as sepsis or septic shock, where an infection spreads through the bloodstream and organs start to shut down.

Other cholangitis complications can include liver failure, kidney problems, and pus-filled masses in the liver. Gallbladder inflammation, known as cholecystitis, and inflammation of the pancreas, called pancreatitis, are other potential cholangitis complications, as is multiple organ failure. A blood clot blockage of the liver’s main blood vessel, known as portal vein thrombosis, also may occur.

In the absence of treatment, mortality rates from acute cholangitis are as high as 50%. However, with appropriate medical treatment, cholangitis usually can be managed or resolved entirely. As such, prompt diagnosis and treatment are vital for the prevention of serious complications.

Living with cholangitis

In cases of acute cholangitis, prompt medical treatment is required and can usually resolve the condition entirely. But chronic types such as autoimmune cholangitis are usually lifelong and frequently necessitate long-term changes in day-to-day routines. In either case, it’s absolutely essential for patients to communicate openly with their healthcare team and follow their instructions, such as taking medications as directed. Prevention, when feasible, is typically the best cholangitis treatment.

Seeking medical care at the first sign of liver disease-related symptoms can help to secure a cholangitis diagnosis and get a patient started on treatment.

Chronic health conditions tend to have a negative impact on a person’s emotional well-being. Therefore, ensuring proper support in terms of mental health is also an important factor when living with cholangitis.

It’s generally recommended that people with chronic cholangitis avoid exposure to drugs and toxins that can stress the liver, including alcohol and other recreational drugs, as well as exposure to smoke and environmental chemicals.

Eating a well-balanced diet, and particularly limiting intake of saturated fats and sugars, is important for managing nutrition when living with cholangitis, as is taking any recommended supplements. Similarly, getting regular exercise and staying physically active can be an important part of disease management.

The American Liver Foundation offers a list of online support resources for those affected by a liver condition, including cholangitis. The PBCers Organization is focused on education and support for the primary biliary cholangitis patient community.

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