Cholangitis

Author: Adam J Rosh, MD, Assistant Professor, Department of Emergency Medicine, Wayne
State University/Detroit Receiving Hospital
Coauthor(s): Jeffrey A Manko, MD, Assistant Professor of Emergency Medicine, Director,
Emergency Medicine Residency Program, Consulting Staff, Emergency Medicine Services, New
York University/Bellevue Medical Center; Sally Santen, MD, Program Director, Assistant
Professor, Department of Emergency Medicine, Vanderbilt University
Contributor Information and Disclosures

Updated: Jun 11, 2010

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Introduction
Background

Acute cholangitis is a bacterial infection superimposed on an obstruction of the biliary tree most
commonly from a gallstone, but it may be associated with neoplasm or stricture.

Pathophysiology

The main factors in the pathogenesis of acute cholangitis are biliary tract obstruction, elevated
intraluminal pressure, and infection of bile. A biliary system that is colonized by bacteria but is
unobstructed, typically does not result in cholangitis. It is believed that biliary obstruction
diminishes host antibacterial defenses, causes immune dysfunction, and subsequently increases
small bowel bacterial colonization. Although the exact mechanism is unclear, it is believed that
bacteria gain access to the biliary tree by retrograde ascent from the duodenum or from portal
venous blood. As a result, infection ascends into the hepatic ducts, causing serious infection.
Increased biliary pressure pushes the infection into the biliary canaliculi, hepatic veins, and
perihepatic lymphatics, leading to bacteremia (25-40%). The infection can be suppurative in the
biliary tract.

The bile is normally sterile. In the presence of gallbladder or common duct stones (CBD),
however, the incidence of bactibilia increases. The most common organisms isolated in bile are
Escherichia coli (27%), Klebsiella species (16%), Enterococcus species (15%), Streptococcus
species (8%), Enterobacter species (7%), and Pseudomonas aeruginosa (7%). Organisms
isolated from blood cultures are similar to those found in the bile. The most common pathogens
isolated in blood cultures are E coli (59%), Klebsiella species (16%), Pseudomonas aeruginosa
(5%), and Enterococcus species (4%). In addition, polymicrobial infection is commonly found in
bile cultures (30-87%) and less frequent in blood cultures (6-16%). For related pathophysiology,
please see the Cholelithiasis and Cholecystitis and Biliary Colic articles.

Primary sclerosing cholangitis is a chronic liver disease that is thought to be due to an

autoimmune mechanism.1 It is characterized by inflammation and fibrosis of the intrahepatic and
extrahepatic bile ducts. This condition ultimately leads to portal hypertension and cirrhosis of the
liver with the only definitive treatment being a liver transplant.2  For more on this condition,
please refer to the Primary Sclerosing Cholangitis article.

Frequency

United States

Cholangitis is relatively uncommon. It occurs in association with other diseases that cause biliary
obstruction and bactibilia (eg, after endoscopic retrograde cholangiopancreatography [ERCP], 1-
3% of patients develop cholangitis). Risk is increased if dye is injected retrograde.

International

Recurrent pyogenic cholangitis, sometimes referred to as Oriental cholangiohepatitis, is endemic

to Southeast Asia. It is characterized by multiple occurrences of biliary tract infection,
intrahepatic and extrahepatic biliary stone formation, hepatic abscesses, and dilatation and
stricturing of the intrahepatic and extrahepatic bile duct.3 For more on this condition, please refer
to the Recurrent Pyogenic Cholangitis article.

Mortality/Morbidity

 Mortality of cholangitis is high due to the predisposition in people with underlying

disease. Historically, the mortality rate was 100%. With the advent of endoscopic
retrograde cholangiography, therapeutic endoscopic sphincterotomy, stone extraction,
and biliary stenting, the mortality rate has significantly declined to approximately 5-10%.
  The following patient characteristics are associated with higher morbidity and mortality
rates:
o Hypotension
o Acute renal failure
o Liver abscess
o Cirrhosis
o Inflammatory bowel disease
o High malignant strictures
o Radiologic cholangitis – Post percutaneous transhepatic cholangiography
o Female gender
o Age older than 50 years
o Failure to respond to antibiotics and conservative therapy
 Advanced age, concurrent medical problems, and delay in decompression increase the
emergent operative mortality rate (17-40%).
 The mortality rate of elective surgery after medical stabilization is significantly less
(approximately 3%).
 In the past, suppurative cholangitis was thought to have increased morbidity; however,
prospective studies have not found this to be true.

Race

 Cholangitis frequently occurs secondary to a gallstone obstructing the common bile duct.
Therefore, it carries the same risk factors as that of cholelithiasis.
 Prevalence of gallstones is highest in fair-skinned people of Northern European descent
as well as in Hispanic populations, Native Americans, and Pima Indians.
 In addition, certain Asian populations and inhabitants of countries where intestinal
parasites are common are also at increased risk. Asians are more likely to have primary
stones due to chronic biliary infections, parasites, bile stasis, and biliary strictures.
Recurrent pyogenic cholangitis (Oriental cholangiohepatitis) rarely is observed in the
United States
 African Americans with sickle cell disease are at increased risk.

Sex

 Although gallstones are more common in women than in men, the male-to-female ratio is
equal in cholangitis.

Age

 Elderly patients are more likely to progress from asymptomatic gallstones to serious
complications of gallstones and cholangitis.
 Suspect cholangitis in older patients presenting with sepsis and mental status changes.
Elderly patients are more prone to gallstones and CBD stones and, therefore, cholangitis.
 The median age at presentation is between 50 and 60 years.
Clinical
History

In 1877, Charcot described cholangitis as a triad of findings of right upper quadrant (RUQ) pain,
fever, and jaundice. The Reynolds pentad adds mental status changes and sepsis to the triad. A
spectrum of cholangitis exists, ranging from mild symptoms to fulminant overwhelming sepsis.
With septic shock, the diagnosis can be missed in up to 25% of patients. Consider cholangitis in
any patient who appears septic, especially in patients who are elderly, jaundiced, or who have
abdominal pain. A history of abdominal pain or symptoms of gallbladder colic may be a clue to
the diagnosis.

 Charcot's triad consists of fever, RUQ pain, and jaundice. It is reported in up to 50-70%
of patients with cholangitis. However, recent studies believe it is more likely to be
present in 15-20% of patients.
 Fever is present in approximately 90% of cases.
 Abdominal pain and jaundice is thought to occur in 70% and 60% of patients,
respectively.
 Patients present with altered mental status 10-20% of the time and hypotension
approximately 30% of the time. These signs, combined with Charcot's triad, constitute
Reynolds pentad.
 Consequently, many patients with ascending cholangitis do not present with the classic
signs and symptoms.4
 Most patients complain of RUQ pain; however, some patients (ie, elderly persons) are too
ill to localize the source of infection.
 Other symptoms include the following:
o Jaundice
o Fever, chills, and rigors
o Abdominal pain
o Pruritus
o Acholic or hypocholic stools
o Malaise
 The patient's medical history may be helpful. For example, a history of the following
increases the risk of cholangitis:
o Gallstones, CBD stones
o Recent cholecystectomy
o Endoscopic manipulation or ERCP, cholangiogram
o History of cholangitis
o History of HIV or AIDS: AIDS-related cholangitis is characterized by
extrahepatic biliary edema, ulceration, and obstruction. The etiology is uncertain,
but it may be related to cytomegalovirus or Cryptosporidium infections. The
management of this condition is described below, although decompression is
usually not necessary.
Physical

 In general, patients with cholangitis are quite ill and frequently present in septic shock
without an apparent source of the infection.
 Physical examination may reveal the following:
o Fever (90%), although elderly patients may have no fever
o RUQ tenderness (65%)
o Mild hepatomegaly
o Jaundice (60%)
o Mental status changes (10-20%)
o Sepsis
o Hypotension (30%)
o Tachycardia
o Peritonitis (uncommon, and should lead to a search for an alternative diagnosis)

Causes

In Western countries, choledocholithiasis is the most common cause of acute cholangitis,

followed by ERCP and tumors.

Any condition that leads to stasis or obstruction of bile in the CBD, including benign or
malignant stricture, parasitic infection, or extrinsic compression by the pancreas, can result in
bacterial infection and cholangitis. Partial obstruction is associated with a higher rate of infection
than complete obstruction.

 CBD stones predispose patients to cholangitis.

o Approximately 10-15% of patients with cholecystitis have CBD stones.
o Approximately 1% of patients post cholecystectomy have retained CBD stones.
Most CBD stones are immediately symptomatic, while some remain
asymptomatic for years.
o Some CBD stones are formed primarily rather than secondarily to gallstones.
 Obstructive tumors cause cholangitis. Partial obstruction is associated with an increased
rate of infection compared with that of complete neoplastic obstruction.
o Pancreatic cancer
o Cholangiocarcinoma5
o Ampullary cancer
o Porta hepatis tumors or metastasis
 Additional causes of cholangitis include the following:
o Strictures or stenosis
o Endoscopic manipulation of the CBD
o Choledochocele
o Sclerosing cholangitis (from biliary sclerosis)
o AIDS cholangiopathy
o Ascaris lumbricoides infections
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Primary Sclerosing Cholangitis

Medical Author: Dennis Lee, MD
Medical Editor: Jay W. Marks, MD

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
 How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Find a local Gastroenterologist in your town

What is primary sclerosing cholangitis (PSC)?

Primary sclerosing cholangitis (PSC) is a chronic (lasting years and decades), progressive
(worsening over time) disease of the bile ducts that channel bile from the liver into the intestines.

The liver performs many functions; one of them is manufacturing bile. Bile is a watery liquid
made by the cells of the liver that is important for digesting food in the intestine, particularly fat,
and ridding the body of toxins. Liver cells secrete the bile they make into small canals within the
liver. The bile flows through the canals and into larger collecting canals (ducts) within the liver
(the intrahepatic bile ducts). The bile then flows within the intrahepatic bile ducts out of the liver
and into the extrahepatic bile ducts. From the extrahepatic bile ducts, the bile flows into the
intestine where the bile mixes with food.

In primary sclerosing cholangitis, the intrahepatic and extrahepatic bile ducts become inflamed,
scarred and thickened (sclerotic), narrowed, and finally obstructed. Obstruction of the ducts can
lead to abdominal pain, itching, jaundice, infection in the bile ducts (cholangitis), and liver
scarring that leads to liver cirrhosis and liver failure.

Primary Sclerosing Cholangitis (cont.)

In this Article

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
  How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Primary Sclerosing Cholangitis Glossary
 Primary Sclerosing Cholangitis Index
 Find a local Gastroenterologist in your town

How common is primary sclerosing cholangitis?

Primary sclerosing cholangitis is a rare disease with an estimated prevalence in the United States
of 6 per 100,000 people. It is more common in men then in women; approximately 70% of
primary sclerosing cholangitis patients are men. The mean age at diagnosis of primary sclerosing
cholangitis is around 40 years of age.

There is a strong association between primary sclerosing cholangitis and chronic ulcerative
colitis. Primary sclerosing cholangitis can also occur alone or in association with Crohn's
disease, a disease of the intestines that is related to ulcerative colitis.

What causes primary sclerosing cholangitis?

The cause of primary sclerosing cholangitis is not known. A small subset (approximately 10%)
of primary sclerosing cholangitis patients has a rapidly progressive form of the disease with early
onset of abdominal pain, fever, and itching that responds dramatically to treatment with
corticosteroids. Since corticosteroids (such as prednisone) are medications for treating immune
diseases such as ulcerative colitis, Crohn's disease, and systemic lupus erythematosus, this small
subset of primary sclerosing cholangitis patients are believed to have an immune disorder
causing their primary sclerosing cholangitis.

What are the symptoms of primary sclerosing cholangitis?

Most patients with early primary sclerosing cholangitis have no symptoms, and the presence of
primary sclerosing cholangitis is recognized only because of abnormally elevated blood levels of
liver enzymes (particularly alkaline phosphatase levels) that often are performed along with a
routine physical examination.

Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus).
As the disease progresses, patients may develop jaundice (yellowing of skin and darkening of
urine). Jaundice is due to the accumulation of bilirubin in the body. The bilirubin accumulates
because it is not able to be eliminated in the bile due to prolonged obstruction of the bile ducts.
The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow. The reason for
the pruritus is not entirely known. It may be due to accumulation of bile salts in the body, also as
a result of obstruction of the bile ducts.
As primary sclerosing cholangitis progresses, patients typically develop right upper abdominal
pain, fever, fatigue, pruritus, and jaundice. These patients also are at risk of developing primary
sclerosing cholangitis complications.

The patients with the autoimmune form of primary sclerosing cholangitis have more rapid and
early onset of symptoms of abdominal pain, jaundice and fever than the majority of patients with
the more indolent form of primary sclerosing cholangitis.

Primary Sclerosing Cholangitis (cont.)

In this Article

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
 How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Primary Sclerosing Cholangitis Glossary
 Primary Sclerosing Cholangitis Index
 Find a local Gastroenterologist in your town

What are the complications of primary sclerosing cholangitis?

Cirrhosis

As primary sclerosing cholangitis progresses, the disease causes cirrhosis of the liver
(irreversible scarring of the liver) and liver failure; leading to the consideration of liver
transplantation. Primary sclerosing cholangitis is, in fact, one of the more common reasons for
liver transplantation. Patients with advanced cirrhosis may develop frequent infections, fluid in
the ankles and the abdomen (ascites), internal bleeding from rupture of esophageal varices, and
mental confusion with progression to coma (hepatic encephalopathy).

Cholangitis

The narrowing of the bile ducts predisposes the bile to bacterial infection (cholangitis).
Cholangitis is a serious and potentially life-threatening infection with fever, shaking chills
(rigors), jaundice, and upper abdominal pain. Cholangitis can result in bacterial infection
spreading to the blood stream (a condition called sepsis). Sepsis can cause damage to kidneys
and lungs and even cause shock.
Cholangiocarcinoma

Cholangiocarcinoma (cancer of the bile ducts) is more common among patients with primary
sclerosing cholangitis. An estimated 9%-15% of patients with primary sclerosing cholangitis will
develop cholangiocarcinoma, a very lethal type of cancer. Patients at highest risks for developing
cholangiocarcinoma are primary sclerosing cholangitis patients with cirrhosis who also have
long-standing ulcerative colitis.

Colon cancer

Long-standing chronic ulcerative colitis alone is a risk factor for colon cancer. Patients with both
primary sclerosing cholangitis and ulcerative colitis have an even higher risk for developing
colon cancer than patients with ulcerative colitis alone.

Primary Sclerosing Cholangitis (cont.)

In this Article

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
 How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Primary Sclerosing Cholangitis Glossary
 Primary Sclerosing Cholangitis Index
 Find a local Gastroenterologist in your town

How is the diagnosis of primary sclerosing cholangitis made?

The diagnosis of primary sclerosing cholangitis is suspected from the symptoms (such as fatigue,
itching, and jaundice) and abnormal laboratory tests (such as abnormally elevated blood levels of
alkaline phosphatase and other liver enzymes); and is confirmed by demonstration of abnormally
thickened bile ducts using special radiological tests. It is also important to exclude other diseases
that can mimic primary sclerosing cholangitis. These diseases include primary biliary cirrhosis
(PBC), gallstones in the bile ducts, bile duct cancers and strictures.

Blood tests

The blood level of alkaline phosphates is usually elevated in primary sclerosing cholangitis. The
blood levels of other liver enzymes (AST and ALT) may also be mildly elevated. Except in those
patients with the autoimmune form of primary sclerosing cholangitis, the bilirubin usually is
normal but gradually increases as the disease progress. Antimitochondrial antibody (AMA),
which is elevated in patients with PBC, is usually normal in patients with primary sclerosing
cholangitis.

Radiologic tests

Endoscopic retrograde cholangio-pancreatography (ERCP) and magnetic resonance cholangio-

pancreatography (MRCP) are commonly performed to visualize the intrahepatic and extrahepatic
ducts. These ducts are typically normal in appearance in patients with PBC, but in primary
sclerosing cholangitis patients, these ducts have a beaded appearance (multiple narrowings along
the ducts with areas of widening in between).

MRCP is noninvasive and safe. ERCP is more invasive and carries a 5%-6% chance of causing
an attack of acute pancreatitis. However, ERCP has the advantage of obtaining cell samples (a
process called brush cytology) from the bile ducts. Brush cytology is not very accurate, but
sometimes can help to diagnose cholangiocarcinoma. Also, during ERCP, doctor can also insert
balloons and stents across major areas of narrowing (dominant strictures) to relieve obstruction
and treat infection.

Colonoscopy

Patients with primary sclerosing cholangitis have a high likelihood of also having ulcerative
colitis, and, as mentioned previously, patients with primary sclerosing cholangitis and
ulcerative colitis have higher risks of developing colon cancer. Thus, colonoscopy is
important to both diagnPrimary Sclerosing Cholangitis (cont.)
In this Article

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
 How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Primary Sclerosing Cholangitis Glossary
 Primary Sclerosing Cholangitis Index
 Find a local Gastroenterologist in your town

How is primary sclerosing cholangitis treated?

The treatment of primary sclerosing cholangitis includes:

 cholestyramine (Questran) or rifampin (Rifadin) to diminish itching

 Antibiotics for infection, specifically cholangitis

 Vitamin D and calcium to prevent bone loss (osteoporosis)

 Medications such as ursodiol (UDCA) to retard the progression of primary sclerosing

cholangitis

 ERCP with balloon dilatation and/or stenting (a procedure in which the bile ducts are
stretched open) for primary sclerosing cholangitis patients with dominant extra-hepatic
duct strictures

 Liver transplantation for patients with advanced cirrhosis

Medications

Many medications [such as ursodiol (UDCA), prednisone, methotrexate (Rheumatrex, Trexall),

colchicine, 6-mercaptopurine, tacrolimus (Prograf), cyclosporine (Neoral, Gengraf)] have been
studied to treat primary sclerosing cholangitis. Except in the case of prednisone for autoimmune
form of primary sclerosing cholangitis, none of the other medications have shown a consistent
benefit on survival or decreased need for liver transplantation.

UDCA

Ursodiol (UDCA) is a bile acid that is given orally and replaces other bile acids in the body.
UDCA is believed to protect against damaging effects of other bile acids on the liver cells and
also induce formation of antioxidants. UDCA is the most extensively studied medication for
primary sclerosing cholangitis. At standard doses (15 mg/kg/day), it has been shown to improve
fatigue, and improve blood levels of liver enzymes in patients with primary sclerosing
cholangitis. In higher doses (20-30 mg/kg/day) UDCA has been shown to improve blood liver
enzymes, decrease bile duct inflammation, and decrease liver scarring. However, there is still no
conclusive evidence that UDCA actually prolongs life or decreases the need for liver
transplantation in primary sclerosing cholangitis patients. Doctors are currently awaiting the
results of a multi-center, placebo controlled trial of high dose UDCA in primary sclerosing
cholangitis.

Meanwhile, most doctors are treating primary sclerosing cholangitis patients with high dose
UDCA (20-30 mg/kg/day) since UDCA is safe and well tolerated, and it may improve bile duct
disease and retard the development of liver cirrhosis. Moreover, at least one study showed that
UDCA may decrease colon cancer risk in patients with both primary sclerosing cholangitis and
chronic ulcerative colitis.

Treatment of dominant strictures

Dominant strictures are major narrowings in the extrahepatic bile ducts. Dominant strictures of
the extrahepatic bile ducts occur in 7%-20% of primary sclerosing cholangitis patients. In
selected primary sclerosing cholangitis patients with dominant strictures, ERCP and balloon
dilatation (stretching) of the stricture can improve symptoms and abnormal blood levels of liver
enzymes and bilirubin. Some doctors also believe that successful dilatation of dominant
strictures decreases the risk of developing cholangitis. However, ERCP and dilatation of
dominant strictures should be done in centers with highly experienced physicians. During ERCP,
doctors often also perform brush cytology of the dominant strictures to exclude
cholangiocarcinoma.

Surgery is another treatment for dominant extrahepatic strictures in primary sclerosing

cholangitis patients. In carefully selected patients, surgical resection of the stricture followed by
creation of a choledocho-jejunostomy (an artificial passage for bile formed by attaching the bile
duct from above the stricture directly into the small intestine) can improve symptoms, delay liver
transplantation, and lower the risk of cholangiocarcinoma. However, few surgeons recommend
surgical resection of dominant strictures because they are concerned that scarring around the
liver from such surgery may complicate future liver transplantation.

Liver transplantation

Even with modern management, most primary sclerosing cholangitis patients will die within 10
years of the time of diagnosis without liver transplantation. Transplantation is now the definitive
treatment in primary sclerosing cholangitis patients with advanced cirrhosis and liver failure.
One year survival following transplantation is 85%-90%, and five year survival is as high as
85%. Reasons for liver transplant in primary sclerosing cholangitis patients are similar to those
in other forms of end-stage liver disease. They are:

 Internal bleeding due to rupture of esophageal varices

 Severe ascites that are refractory to medical treatment

 Frequent episodes of bacterial cholangitis

 Hepatic encephalopathy

The Mayo clinic devised a scoring model to help doctors predict the life span of primary
sclerosing cholangitis patients not having a liver transplant. This model includes age, blood
levels of bilirubin, albumin, AST and a history of bleeding from esophageal varices. The model
suggests that when a patient's score estimates 6 month-survival is less than 80%, the patient
should be considered for liver transplantation.

ose ulcerative colitis and for early detection of cancer or precancerous conditions.

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 Primary Sclerosing Cholangitis Index

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 Find a Local Doctor

Primary Sclerosing Cholangitis (cont.)

In this Article

 What is primary sclerosing cholangitis (PSC)?

 How common is primary sclerosing cholangitis?
 What causes primary sclerosing cholangitis?
 What are the symptoms of primary sclerosing cholangitis?
 What are the complications of primary sclerosing cholangitis?
 How is the diagnosis of primary sclerosing cholangitis made?
 How is primary sclerosing cholangitis treated?
 Primary Sclerosing Cholangitis At A Glance
 Primary Sclerosing Cholangitis Glossary
 Primary Sclerosing Cholangitis Index
 Find a local Gastroenterologist in your town

Primary Sclerosing Cholangitis At A Glance

 Primary sclerosing cholangitis is a chronic, progressive disease of the liver.

 The cause of primary sclerosing cholangitis is not known though an immune cause is suspected
in at least a minority of patients.

 Primary sclerosing cholangitis has a strong association with ulcerative colitis and cancer of the
bile ducts.

 The primary symptoms of primary sclerosing cholangitis are due to obstruction of the bile ducts
and cirrhosis of the liver.

 Primary sclerosing cholangitis is diagnosed on the basis of abnormal blood tests and radiologic
imaging of the bile ducts.

 Primary sclerosing cholangitis is treated with medications, endoscopy, and liver transplantation.