Cholangitis treatment and management options
Last updated Aug. 16, 2024, by Marisa Wexler, MS
Fact-checked by Marta Figueiredo, PhD
Cholangitis is a form of inflammation that affects the bile duct system, a set of tubes that are used to move bile, a digestive fluid, from the liver to the gallbladder and intestines. Bile is a sticky, yellow-green fluid produced by the liver, stored in the gallbladder, and released into the small intestine to help the body digest certain fats and proteins.
Depending on its course and underlying cause, cholangitis can be classified as either acute or chronic, and as either primary or secondary.
Understanding cholangitis
Acute cholangitis refers to a form of the disease that develops suddenly. About 65% of cases are estimated to be caused by gallstones, or hardened bile deposits that can form in the gallbladder. Gallstones can become stuck in the bile ducts, obstructing the fluid’s flow. The stalled bile typically acts as a fertile breeding ground for infectious bacteria, ultimately triggering inflammation.
Chronic cholangitis develops gradually over a fairly long course of time, and symptoms often don’t become apparent until the disease has already progressed to causing organ damage. While it’s usually the result of an autoimmune disease, chronic cholangitis also can occur due to physical injuries in or around the bile ducts.
In addition to these two types, cholangitis also can be classified as primary — when no other cause for bile duct inflammation is identified — or as secondary, when it’s clearly caused by another condition, such as infections or injury.
All forms of cholangitis can cause symptoms such as abdominal pain, fever, and jaundice, marked by yellowed skin and eyes.
In general, all types of cholangitis treatment broadly focus on addressing the underlying cause of bile duct inflammation and restoring flow through the ducts, thereby reducing bile buildup.
Cholangitis can usually be cured or managed, but without treatment the condition can cause fatal complications including liver failure and sepsis, in which an infection spreads through the bloodstream and organs start to fail. As such, prompt diagnosis and rapid treatment are critical for managing all forms of cholangitis.
Acute cholangitis treatment
Also known as ascending cholangitis, acute cholangitis is almost always marked by both a bacterial infection and blocked flow of bile. Biliary obstruction can increase the pressure and affect tissue integrity of the ducts, allowing bacteria to enter the bloodstream.
Acute cholangitis treatment therefore usually has two broad goals: eliminating the infectious bacteria and draining out excess bile. To prevent serious complications, it’s important for treatment to be initiated as soon as is possible.
Antibiotics, or medications that kill bacteria, are a mainstay treatment for acute cholangitis. Typically, antibiotics are given directly into the bloodstream along with fluids. It’s generally recommended to use antibiotics that are good at getting into the liver and are effective for killing a wide range of bacteria. Common antibiotics for cholangitis include:
- imipenem-cilastatin (sold under the brand name Primaxin, among others)
- meropenem (sold as Merrem and other brand names)
- amikacin (sold under the brand name Amikin and others)
- cefepime (sold as Maxipime, among others)
- ceftriaxone (sold under brand names including Ceftrisol Plus and Rocephin)
- gentamicin (sold as Cidomycin, Genticyn, and Garamycin, among others)
- piperacillin-tazobactam (sold as Tazocin and Zosyn, among others)
- levofloxacin (sold under the brand name Levaquin, among others).
Alongside antibiotics to address the infection, ascending cholangitis treatment usually involves biliary drainage and decompression procedures — that is, surgical techniques to remove excess built-up bile from the bile ducts and help get bile flowing normally.
The gold standard technique for bile duct blockage treatment is endoscopic retrograde cholangiopancreatography or ERCP. In this minimally-invasive procedure, X-ray imaging is combined with the insertion of an endoscope — a long, flexible tube with a light source and a video camera — down the mouth and throat, through the stomach, to the first part of the small intestine.
The endoscope allows the observation of the liver and bile duct system, as well as the insertion of other instruments through the tube that can then be used to inject dyes visible on an X-ray. Such tools and instruments also can be used to remove excess bile or blockages like gallstones.
ERCP also can be used to place stents, or thin, hollow tubes that can help prop open the bile ducts, in patients with biliary strictures, or abnormal narrowing of the bile ducts.
According to recommendations from the American Society for Gastrointestinal Endoscopy (ASGE), ERCP should be performed within 48 hours of hospital admission in cases of suspected acute cholangitis.
Endoscopic ultrasound-guided drainage is another form of endoscopic biliary drainage that uses ultrasound to guide the procedure.
More invasive procedures may be used in more severe cholangitis cases, including percutaneous transhepatic cholangiography (PTC) and open surgery. PTC, guided by imaging scans, involves inserting a thin tube called a catheter through the skin of the abdomen into the bile ducts to drain a blocked bile duct. According to the ASGE recommendations, PTC is considered the best option for acute cholangitis treatment when ERCP isn’t feasible or doesn’t work properly.
Chronic cholangitis treatment
Chronic forms of cholangitis are typically associated with autoimmune conditions characterized by abnormal, persistent immune attacks against healthy cells in the bile ducts. Such attacks result in bile duct inflammation and damage, causing bile to build up in the liver. That can ultimately lead to cirrhosis, or severe scarring of the liver, and liver failure.
As such, in contrast with acute forms of cholangitis — which can typically be resolved with prompt treatment — chronic cholangitis often requires lifelong care. Many of the same techniques used for acute cholangitis management, including biliary drainage procedures, also may be used in chronic cholangitis and repeated as needed over time.
Given that chronic types of cholangitis aren’t generally associated with bacterial infections, antibiotics are not standard treatment for these forms of the disease. However, a few small studies have suggested that certain antibiotics may have beneficial effects in some forms of autoimmune cholangitis, possibly by altering levels of bacteria that normally reside in the gastrointestinal system.
Chronic forms of cholangitis may make it harder for the body to absorb certain nutrients and vitamins, so supplements may be given to ensure patients can get adequate nutrition.
People with chronic cholangitis also are recommended to not start or quit smoking, avoid alcohol, and stop using any medications that are known to be toxic to the liver. In addition, chronic cholangitis patients are recommended to maintain a healthy weight, which may help to prevent disease progression.
In severe cases, where medication no longer controls chronic cholangitis and the liver begins to fail, patients may be put on the liver transplant waiting list. A liver transplant refers to the implantation of a healthy liver from a donor into the patient’s body, replacing the diseased organ.
Primary biliary cholangitis treatment
Primary biliary cholangitis or PBC — previously known as primary biliary cirrhosis — is a type of chronic cholangitis characterized by inflammation of the small bile ducts in the liver. Predominantly affecting women, PBC is the most common liver disease linked to chronic cholestasis in adults, and is considered an autoimmune condition.
Standard primary biliary cholangitis treatment consists of ursodiol or ursodeoxycholic acid (UDCA), the only approved first-line therapy for PBC. It’s sold under the brand names Urso and Actigall.
Ursodiol is a nontoxic bile acid that can help get bile flowing through the liver. It has been shown to prevent further liver damage and improve survival among patients, especially when started early in the disease course. Treatment with this bile acid also may delay or eliminate the need for liver transplant. The most common side effects of the medication include abdominal discomfort, abdominal pain, dizziness, hair loss, diarrhea, nausea, and rash.
Ocaliva (obeticholic acid), a molecule that works by activating a bile-regulating liver protein called the farnesoid X receptor, as well as Iqirvo (elafibranor) and Livdelzi (seladelpar) — both modulating the activity of proteins involved in PBC-related processes called PPARs — are second-line PBC therapies. Each of the three may be given in combination with ursodiol to patients not responding to the first-line therapy or alone in those not tolerating ursodiol treatment. Use of Ocaliva is often limited in PBC however, as the therapy can cause worsened liver-related symptoms, including itching.
Given that UDCA, Ocaliva, Iqirvo, and Livdelzi may not be as effective for easing all PBC symptoms, which commonly include itching, other treatments also are used to ease certain disease symptoms.
Primary biliary cholangitis medication that may be used to relieve itchy skin include:
- cholestyramine, sold under brand names including Prevalite and Questran Light, that can help reduce bile acids in the blood
- rifampicin, an antibiotic sold as Rifadin, which is thought to block the brain’s response to itch-inducing chemicals
- fibrates, medications used to lower certain fatty molecules in the blood and sold as Tricor (fenofibrate), Bezalip (bezafibrate), and other brand names
- naloxone (sold under the brand name Evzio, among others), an approved treatment for substance abuse disorders that also can reduce itch
- sertraline, sold under the brand name Zoloft and generic versions, a serotonin reuptake inhibitor that is thought to act on fibers that regulate the perception of itch
- gabapentin (sold under the brand name Neurontin, among others) an anticonvulsant therapy that can relieve itchy feeling.
Current guidelines from the American Association for the Study of Liver Diseases recommend applying a step-by-step treatment approach for relieving itching, also known as pruritus, in people with PBC. This approach may start with cholestyramine. In case the control of pruritus is insufficient with cholestyramine, the guidelines recommend either rifampicin or bezafibrate, with the next option being naloxone; alternative therapies are sertraline and gabapentin.
Cholestyramine is the only approved therapy for the treatment of pruritus associated with PBC. All other medications are used off-label, meaning that the treatment is approved for other medical indications.
Importantly, cholestyramine can change how the body absorbs UDCA, so patients on both medications should never take them at the same time. UDCA should be taken at least one hour before cholestyramine, or four to six hours after taking cholestyramine.
A nondrug approach for itchy skin in PBC patients is phototherapy, in which ultraviolet light is applied to the skin.
Medications able to suppress the immune system, including corticosteroids, also may be prescribed to PBC patients.
In PBC cases where liver function begins to seriously worsen, a liver transplant may be recommended. Liver transplant can usually cure itchiness and other symptoms of PBC, although the disease can still recur in some patients after the transplant.
Several experimental therapies also are being developed for PBC. These include:
- PPAR activators, including seladelpar, saroglitazar, and fenofibrate, which act on pathways contributing to PBC, such as bile acid generation, inflammation, and scarring
- Suppressors of a protein called ileal bile acid transporter, such as linerixibat and volixibat, that may help to reduce bile acid reuptake from the intestines back to the liver and boost bile acid excretion in stools.
Primary sclerosing cholangitis treatment
Primary sclerosing cholangitis (PSC), another type of chronic cholangitis, is typically characterized by inflammation, scarring, and narrowing (or stricturing) of bile ducts both in and outside the liver.
PSC is believed to be associated with autoimmune responses, usually occurring in people with inflammatory bowel disease — a group of autoimmune diseases characterized by inflammation in the intestines. PSC is more common among men and is associated with an increased risk of developing bile duct cancer.
Unlike UDCA for PBC, there currently is no treatment that can slow or stop PSC-related liver damage from getting worse. Care for PSC commonly involves monitoring of liver function, medications to manage symptoms and complications, and procedures like ERCP and PTC to temporarily prop open the bile ducts and drain excess bile.
As with PBC, itchy skin is a common symptom of PSC, and treatment may include cholestyramine, rifampin, naloxone, or UDCA. People with PSC may experience frequent bouts of bacterial cholangitis that can be treated with antibiotics.
Surgery to reconstruct the biliary system and thereby improve bile flow also may be performed. However, the only way to cure PSC is through a liver transplant. It’s relatively uncommon for PSC to recur after a transplant, though it can happen.
Secondary cholangitis treatment
Secondary cholangitis treatment typically focuses on the disease’s underlying cause, including biliary gallstones, tumors in the biliary system or the liver that have narrowed or blocked bile ducts, or inflammation of the pancreas, known as pancreatitis.
In rare cases, sclerosing cholangitis also can occur secondary to another health problem. In such cases, it’s called secondary sclerosing cholangitis, or SSC. Potential causes include:
- longstanding biliary obstruction
- repeated episodes of bacterial cholangitis
- HIV or human immunodeficiency virus-associated bile duct damage
- surgical trauma to the biliary system
- exposure to toxins or medications, including some chemotherapies
- other autoimmune or inflammatory disorders, such as sarcoidosis and pancreatitis
- genetic disorders, iincluding Caroli disease and cystic fibrosis
- tumors that develop in or spread to the liver.
Besides treatment targeting SSC’s underlying cause — such as antivirals or antibiotics to manage infections, surgery to remove tumors, and anti-inflammatory medications to address autoimmune disorders — disease management generally is similar to that of PSC.
Procedures like ERCP may be used to drain excess bile, and medications may be given to manage specific disease symptoms. If liver damage gets to a serious point, a liver transplant may become necessary. UDCA also may be given to people with SSC, though scant data are available on whether this medication is effective for slowing disease progression in this form of cholangitis.
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