Infectious and primary sclerosing cholangitis

Infectious cholangitis is an infection of the bile ducts that occurs due to bile congestion, which favors colonization along the bile ducts with bacteria.

The term “cholangitis” refers to inflammation of the bile ducts, while the term “sclerosing” refers to hardening and fibrosis of the bile ducts caused by chronic inflammation.

Primary sclerosing cholangitis is a progressive disease that causes liver damage and possibly liver failure.

Sclerosing cholangitis is a disease of the bile ducts in the liver.

In primary sclerosing cholangitis, the intrahepatic and extrahepatic bile ducts become inflamed, scarring, thickening, narrowing and eventually closing.

Occlusion of the ducts can cause abdominal pain, itching, jaundice, biliary tract infections (cholangitis) and liver fibrosis, leading to cirrhosis and liver failure.


Development of infectious cholangitis

The bile is usually sterile. Only if the ductus choledochus is closed and the flow of bile decreases (bile congestion), an infection can occur.
The term liver fever was first used by Charcot in a public account in 1887.
The Charcot triad includes:

  1. Intermittent fever accompanied by chills,
  2. abdominal pain in the upper right quadrant,
  3. Jaundice.

Occlusive acute cholangitis was defined by Reynolds and Dargan in 1959 as a syndrome that leads to lethargy, mental confusion, and shock in addition to fever, jaundice, and abdominal pain caused by bile duct occlusion.
These five symptoms were called Reynolds’ pentade.

The infection can also develop retrograde to the ductus choledochus due to acute gallbladder inflammation or as a result of an examination called endoscopic retrograde cholangiopancreatography (ERCP).
The bacteria that most often cause infection are: Escherichia coli, Klebsiella Spp., Enterobacteria Spp., enterococci and streptococci.

Causes of infectious cholangitis

Cholangitis is an inflammation of the bile ducts.
It is usually caused by a bacterial infection that can occur when the bile duct becomes blocked due to a liver tumor, gallstones, or another condition.

Choledocholithiasis is the most common cause of infectious cholangitis (also known as ascending cholangitis).
Choledocholithiasis is the name given to the presence of stones in the last tract of the bile ducts, which ends in the small intestine.

The infection that causes this condition can also spread to the liver.

What causes primary sclerosing cholangitis?

The cause of primary sclerosing cholangitis is unknown.
A proportion of patients (about 10%) with sclerosing cholangitis have a rapidly progressive form of the disease with abdominal pain, fever and itching, which responds well to treatment with corticosteroids.
Because corticosteroids are indicated in the treatment of autoimmune diseases such as ulcerative colitisCrohn’s disease, and systemic lupus erythematosus, this small subset of patients with sclerosing cholangitis is believed to suffer from an autoimmune disease that causes sclerosing cholangitis.

What are the symptoms of infectious cholangitis?

Symptoms of cholangitis can vary from mild to severe, including:

Signs and symptoms of primary sclerosing cholangitis include:

  • Stomach ache
  • Ague
  • Diarrhoea
  • Fatigue
  • Fever
  • Itch
  • Weight loss
  • Jaundice

Primary sclerosing cholangitis does not necessarily cause symptoms in the initial phase.
In some cases, the only sign of this disorder is an abnormality in the blood test, which indicates higher liver counts.

Complications of infectious cholangitis

Patients with infectious cholangitis may develop liver abscesses and hepatic insufficiency, bacteremia and sepsis caused by Gram-negative bacteria.
Severe acute cholangitis can lead to septic shock, acute renal failure and cardiovascular, neurological, respiratory and hepatic disorders.
Patients who have been treated with percutaneous drainage or endoscopically may develop intra-abdominal or percutaneous bleeding, sepsis, fistula, and bile loss.

What are the complications of sclerosing cholangitis?

As primary sclerosing cholangitis progresses, cirrhosis (irreversible fibrosis of the liver) and liver failure may develop.
Primary sclerosing cholangitis is one of the most common reasons for liver transplantation.
Patients with advanced cirrhosis may develop frequent infections, ankle edema, abdominal fluid retention (ascites), bleeding from rupture of esophageal varices, and mental confusion up to coma (heaptic encephalopathy).

The narrowing of the bile ducts can lead to bacterial infection (cholangitis).
Bacterial infections can spread through the bloodstream (a condition called sepsis).
Sepsis can lead to damage to the kidneys and lungs and even up to hypovolemic shock.

Cholangiocarcinoma (carcinoma of the bile ducts) is most common in patients with primary sclerosing cholangitis.
It is estimated that 9-15% of patients with primary sclerosing cholangitis develop bile duct cancer, a type of cancer with a low survival rate.

Colon cancer
Patients with ulcerative colitis and primary sclerosing cholangitis have a greater risk of developing a colon tumor than patients who have ulcerative colitis alone.

Hepatic insufficiency
The chronic inflammation of the bile ducts in the liver can cause cirrhosis, hepatocellular death, and eventually hepatic insufficiency

Diagnosis of primary sclerosing cholangitis

The examinations for the diagnosis of primary sclerosing cholangitis are:

Blood test
The most common anomaly is high alkaline phosphatase or elevated levels of GGT (gamma-glutamyl transferase).

The levels of transaminases in the blood test may be slightly elevated.
The bilirubin is increased when the disease is in an advanced phase.
As the disease progresses, albumin in the blood and prothrombin time (PTT) exceed normal levels.
Immunoglobulin G (IgG), IgM and globulin fraction levels may be elevated.
Also elevated may be levels of gamma globulinemia, perinuclear anti-neutrophil cytoplasmic antibodies, anti-cardiolipin antibodies and antinuclear antibodies.

The ultrasound examination is the initial examination and can reveal dilations of the bile ducts in the liver as well as changes in the spleen; however, it is insufficient for diagnosis.

Endoscopic retrograde cholangiopancreatography (ERCP) is the most important examination in diagnosis, but it is invasive.
Transhepatic cholangiography may be performed if ERCP cannot be used. However, this is another invasive examination.

Cholangiopancreatography with magnetic resonance imaging (MRCP) is the non-invasive alternative to ERCP, which is increasingly used.
Magnetic resonance can be helpful in ruling out other conditions and evaluating the bile duct system.

Rarely, a liver biopsy is performed, which can help determine the stage of the disease.
A recent technique, transient elastography (fibroscan ®), is a noninvasive method of detecting cirrhosis of the liver in patients with chronic liver disease.

Treatment of primary sclerosing cholangitis and medications

Many drugs have been studied for patients with primary sclerosing cholangitis, but so far there has been no evidence of slowing or halting the progression of liver disease.

Drug treatment for itching
Medications that can help against itching include:

  • Antihistamines, such as diphenhydramine (Benadryl, etc.)
  • Medications that prevent the absorption of bile acids, such as colestyramine (quantalan, etc.)

Drug therapy for infections
Bacterial infections can be common in people with primary sclerosing cholangitis. Usually, the doctor then prescribes antibiotics.

Treatment for bile duct occlusion
Blockages that occur on the bile ducts can be treated with:

  1. Balloon expansion and insertion of a stent.

This procedure is used to open the blockages of the largest bile ducts.

Balloon dilatation is a procedure in which the doctor inserts a fine tube (endoscope) with an inflatable balloon at the tip (balloon catheter) into one of the blocked bile ducts.
When the catheter is positioned correctly, the balloon is inflated.
A cylindrical metal lattice structure, a so-called stent, can be inserted into the bile ducts to keep them open.

  1. Operation of the biliary tract.

In certain situations, occlusions in the bile ducts must be surgically removed.
After removing the blockage, the surgeon connects the remaining bile ducts so that bile can continue to flow through the bile duct.


A liver transplant is the only known treatment to cure primary sclerosing cholangitis.
During liver transplantation, the surgeon removes the diseased liver and replaces it with a donor’s new liver.
Liver transplantation is reserved for people with liver failure or other serious complications of primary sclerosing cholangitis.
Recurrence of primary sclerosing cholangitis after liver transplantation is possible, but rarely occurs.

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