Brief overview
- Symptoms: In acute cholangitis, severe pain in the upper abdomen, often high fever, yellowing of the skin; in autoimmune forms, fatigue, upper abdominal discomfort, jaundice, and severe itching.
- Treatment: in the acute form, antibiotics, removal of gallstones if needed; in the autoimmune forms, medications, possibly liver transplantation
- Causes: In acute cholangitis, a bacterial infection; in the autoimmune forms, a narrowing of the bile ducts due to inflammation triggered by the patient’s own immune system
- Risk factors: Gallstones, narrowing of the bile ducts (for example, after surgery or with tumors)
- Diagnosis: physical examination, blood work, ultrasound, and further imaging if needed
- Course of disease: The acute form usually heals with appropriate therapy, autoimmune forms persist for life, increased risk of cirrhosis of the liver as well as bile duct cancer with PSC
- Prognosis: In the acute form usually good, in the autoimmune forms often shortened life expectancy.
What is cholangitis?
In addition, toxic substances from the body enter the intestine via the liver and bile ducts and are excreted in the stool.
In cholangitis, the bile ducts are inflamed, but the gallbladder is not affected by the inflammation.
Types of cholangitis
Physicians distinguish between an acute bacterial form of cholangitis, which is caused by bacteria rising from the duodenum, and various special forms, which are autoimmune diseases:
Acute bacterial cholangitis
In acute cholangitis, bacteria from the duodenum enter the bile ducts. Under certain circumstances, these settle and trigger an acute, purulent inflammation of the bile ducts. Frequently, those affected suffer from gallstones: These obstruct the outflow of bile and thus favor bacterial colonization. Since overweight women in particular have an increased risk of gallstones after the age of 40, they are twice as likely to develop acute cholangitis as men.
Autoimmune forms of cholangitis
In addition to bacterial cholangitis, there are also special forms of bile duct inflammation that belong to the autoimmune diseases:
- Primary sclerosing cholangitis (PSC) is a chronic progressive inflammation of the bile ducts inside and outside the liver. The disease is closely related to other autoimmune diseases such as the chronic inflammatory bowel disease ulcerative colitis. Men are affected about twice as often as women, usually between the ages of 30 and 50.
- In secondary sclerosing cholangitis (SSC), in contrast to PSC, a specific trigger can almost always be identified, such as inadequate blood supply to the bile ducts (ischemia), injury to the bile ducts due to surgical procedures, or certain infections.
The autoimmune forms of cholangitis lead in the course to scarring (sclerosis) of the bile ducts up to bile stasis (cholestasis). In the later stages, affected individuals often develop cirrhosis of the liver, which can only be treated with liver transplantation.
What are the symptoms of cholangitis?
Acute bacterial cholangitis, PBC and PSC show common symptoms as well as some differences in the clinical picture. In particular, symptoms in the autoimmune forms do not develop suddenly, but rather gradually.
Symptoms of acute (bacterial) cholangitis
The breakdown product (bilirubin) of the red blood pigment (hemoglobin) is no longer excreted via the bile, passes into the blood and is deposited in the skin and mucous membranes. The yellowing of the skin is associated with severe itching in some people.
Symptoms of primary biliary cholangitis
In the early stages, primary biliary cholangitis often causes no symptoms or only nonspecific symptoms such as chronic fatigue and upper abdominal discomfort. Many sufferers also complain of massive, excruciating itching.
Because PBC leads to liver fibrosis and cirrhosis as the disease progresses, signs of liver damage are later added, such as yellowing of the skin and an accumulation of fluid in the abdomen (ascites). Other symptoms of PBC include dyslipidemia, fatty stools, deficiency of fat-soluble vitamins, and, especially in women, recurrent urinary tract infections.
Symptoms of primary sclerosing cholangitis
How is cholangitis treated?
The therapy of cholangitis depends on the cause of the disease.
Bacterial cholangitis
The trigger for acute bile duct inflammation is usually bacteria. The physician therefore usually prescribes high doses of antibiotics to the affected person. In some cases, he also uses a combination of two different classes of antibiotics with different mechanisms of action to cover a wider spectrum of germs (broad-spectrum antibiotics).
People with acute cholangitis are advised not to eat for at least 24 hours to avoid promoting bile flow. In addition, sufferers are given painkillers such as metamizole and antipyretics such as paracetamol and ibuprofen. Usually, the pain subsides after a few days. Sufferers are also advised to drink enough fluids.
Gallstone removal
If cholangitis is due to a blockage of the bile ducts caused by gallstones, it is important to remove them. In some cases, the surgeon will insert a stent into the bile duct. The stent is a tube that keeps the bile duct open and thus improves the outflow of bile into the small intestine.
Primary sclerosing cholangitis
Primary sclerosing cholangitis and primary biliary cholangitis are autoimmune diseases and have not yet been treatable causally. Since affected patients often suffer from jaundice, the therapeutic focus is on the excretion of bile acids. The drug ursodeoxycholic acid not only improves jaundice, but also improves the prognosis of those affected, at least in the case of PBC.
In acute inflammatory episodes in the context of PSC, the physician also uses antibiotics. If there is a deficiency of fat-soluble vitamins, those affected receive appropriate vitamin preparations to counteract deficiency symptoms.
In the course of the disease, PSC and PBC lead to progressive scarring of the liver tissue (cirrhosis). In the final stage of cirrhosis, the last treatment option is liver transplantation.
Causes and risk factors
Acute cholangitis and the autoimmune forms PBC and PSC have very different underlying causes.
Acute cholangitis caused by intestinal bacteria
Acute cholangitis is often caused by intestinal bacteria that migrate from the small intestine via the common bile duct (choledochal duct) into the gallbladder and bile duct system. The common bile duct opens into the duodenum together with the pancreatic duct.
Acute cholangitis due to gallstones (cholelithiasis)
Gallstones are usually caused by excessive cholesterol content in the bile. Overweight women over the age of 40 are particularly at risk and often have gallstones.
If the gallstones move from the gallbladder into the biliary system, they block the bile ducts, whereupon bile backs up – sometimes into the liver. Bacteria in the bile ducts multiply more easily under these conditions. Irritation of the bile duct mucosa then occurs, and purulent inflammation (cholangitis) spreads more quickly.
Narrowing of the bile ducts, for example due to anatomical features, a tumor or surgical procedures in the bile duct area, also increases the risk of cholangitis.
PSC and PBC: autoimmune diseases
Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are special forms of cholangitis that are based on an autoimmune inflammatory process. The bile ducts of those affected are chronically inflamed and narrow in the course of the disease, resulting in a backlog of bile. The reason for the immune system attacking the body’s own tissue is not known.
Examinations and diagnosis
This is followed by a physical examination, during which the doctor takes a closer look at the skin, among other things, if cholangitis is suspected. A possible yellowing of the skin or so-called liver skin signs indicate liver damage. Liver skin signs are typical skin changes that occur in chronic liver disease. These include, for example, star-shaped dilatations of skin vessels (spider naevi), reddening of the palms (palmar erythema) and very red, smooth, varnish-like shiny lips (varnish lips).
The physician also listens to the abdomen with a stethoscope to check for bowel sounds and intestinal air and stool content. He also palpates the abdomen. The doctor often presses under the right ribcage and asks the patient to take a deep breath. If the pain increases and the patient reflexively stops breathing, the suspicion of inflammation is confirmed. During the palpation, the doctor also checks the liver and spleen, which are often enlarged in PBC.
Since cholangitis can only be detected to a limited extent by the physical examination, further tests usually follow:
Blood test
In PBC and PSC, the so-called cholestasis parameters are often elevated. These are laboratory values that indicate bile stasis, for example total bilirubin and alkaline phosphatase (AP). In PBC, elevated liver and cholesterol levels (hypercholesterolemia) may occur later in the course of the disease.
In addition, because PBC is an autoimmune disease, levels of specific autoantibodies (AMA-M2 and PBC-specific ANA) are elevated. These are antibodies that specifically target the body’s own structures. Specific PSC auto-antibodies, on the other hand, are not known; however, the so-called ANCA, the antineutrophil cytoplasmic antibodies, are elevated in many affected individuals.
Ultrasound (sonography)
Ultrasound examination of the abdomen (abdominal ultrasonography) may provide initial indications of the cause of cholangitis. Dilated bile ducts indicate a biliary obstruction. If gallstones are present in the bile duct system, they usually form in the gallbladder and are best visualized there.
Further imaging
If primary sclerosing cholangitis is suspected, magnetic resonance cholangiopancreatography (MRCP) is considered a reliable diagnostic tool; in some cases, endoscopic retrograde cholangiopancreatography (ERCP) is also used.
ERCP enables imaging of the bile ducts from the inside. The doctor inserts a thin tube through the esophagus and stomach into the duodenum, through which he injects X-ray contrast medium into the common bile duct and pancreatic duct. He then uses an X-ray machine to X-ray the bile duct system. If gallstones can be detected, they are removed directly during the examination.
Course of the disease and prognosis
Provided that the acute cholangitis heals and the doctor removes any gallstones present, the prognosis for bile duct inflammation is very good. For most of those affected, it then remains a one-off illness.
In acute bacterial cholangitis, treatment with antibiotics is very important to prevent the bacteria from spreading throughout the body via the bloodstream and leading to blood poisoning (cholangiosepsis). In advanced stages, cholangitis may spread to the rest of the liver tissue and cause purulent abscesses.
The longer cholangitis persists, the higher the risk for narrowing (stricture) and scarring of the bile ducts. Narrowing of the bile ducts prevents the unrestricted outflow of bile and increases the risk of bile reflux.
While acute cholangitis is not associated with a reduction in life expectancy, life expectancy is reduced in PSC and PBC. For example, the five-year survival rate for people with symptomatic PBC is about 50 percent (for those without symptoms, it is 90 percent). Median survival for PSC without liver transplantation is about ten to 20 years from the time of diagnosis.
