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

and perihepatic lymphatics. stone extraction. however. As a result. the incidence of bactibilia increases. The most common organisms isolated in bile are Escherichia coli (27%). Pseudomonas aeruginosa (5%). Klebsiella species (16%). . hepatic veins. For related pathophysiology. With the advent of endoscopic retrograde cholangiography. Historically. The bile is normally sterile. infection ascends into the hepatic ducts. polymicrobial infection is commonly found in bile cultures (30-87%) and less frequent in blood cultures (6-16%). sometimes referred to as Oriental cholangiohepatitis. The infection can be suppurative in the biliary tract. and Enterococcus species (4%). Increased biliary pressure pushes the infection into the biliary canaliculi. is endemic to Southeast Asia. It occurs in association with other diseases that cause biliary obstruction and bactibilia (eg. the mortality rate was 100%. Organisms isolated from blood cultures are similar to those found in the bile. and Pseudomonas aeruginosa (7%). In addition. please see the Cholelithiasis and Cholecystitis and Biliary Colic articles. It is characterized by multiple occurrences of biliary tract infection. therapeutic endoscopic sphincterotomy. intrahepatic and extrahepatic biliary stone formation. Although the exact mechanism is unclear. This condition ultimately leads to portal hypertension and cirrhosis of the liver with the only definitive treatment being a liver transplant. causing serious infection. Streptococcus species (8%). and biliary stenting. Mortality/Morbidity y Mortality of cholangitis is high due to the predisposition in people with underlying disease.3 For more on this condition. International Recurrent pyogenic cholangitis. please refer to the Primary Sclerosing Cholangitis article. Primary sclerosing cholangitis is a chronic liver disease that is thought to be due to an autoimmune mechanism. the mortality rate has significantly declined to approximately 5-10%. please refer to the Recurrent Pyogenic Cholangitis article. Risk is increased if dye is injected retrograde.small bowel bacterial colonization. hepatic abscesses. 13% of patients develop cholangitis). after endoscopic retrograde cholangiopancreatography [ERCP].2 For more on this condition. In the presence of gallbladder or common duct stones (CBD). and dilatation and stricturing of the intrahepatic and extrahepatic bile duct. Enterococcus species (15%). leading to bacteremia (25-40%). The most common pathogens isolated in blood cultures are E coli (59%). Enterobacter species (7%).1 It is characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. Klebsiella species (16%). Frequency United States Cholangitis is relatively uncommon. it is believed that bacteria gain access to the biliary tree by retrograde ascent from the duodenum or from portal venous blood.

the male-to-female ratio is equal in cholangitis. concurrent medical problems. Elderly patients are more prone to gallstones and CBD stones and. and biliary strictures. and Pima Indians. Prevalence of gallstones is highest in fair-skinned people of Northern European descent as well as in Hispanic populations. Asians are more likely to have primary stones due to chronic biliary infections. Sex y Although gallstones are more common in women than in men. . The mortality rate of elective surgery after medical stabilization is significantly less (approximately 3%). cholangitis. Therefore. and delay in decompression increase the emergent operative mortality rate (17-40%). it carries the same risk factors as that of cholelithiasis. bile stasis. Suspect cholangitis in older patients presenting with sepsis and mental status changes. therefore. The median age at presentation is between 50 and 60 years. parasites. In addition. Race y y y y Cholangitis frequently occurs secondary to a gallstone obstructing the common bile duct.y y y y 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. suppurative cholangitis was thought to have increased morbidity. however. Age y y y Elderly patients are more likely to progress from asymptomatic gallstones to serious complications of gallstones and cholangitis. In the past. certain Asian populations and inhabitants of countries where intestinal parasites are common are also at increased risk. Recurrent pyogenic cholangitis (Oriental cholangiohepatitis) rarely is observed in the United States African Americans with sickle cell disease are at increased risk. prospective studies have not found this to be true. Native Americans.

especially in patients who are elderly. cholangiogram o History of cholangitis o History of HIV or AIDS: AIDS-related cholangitis is characterized by extrahepatic biliary edema. ranging from mild symptoms to fulminant overwhelming sepsis. or who have abdominal pain. constitute Reynolds pentad. but it may be related to cytomegalovirus or Cryptosporidium infections. and rigors o Abdominal pain o Pruritus o Acholic or hypocholic stools o Malaise The patient's medical history may be helpful. RUQ pain. some patients (ie. Consider cholangitis in any patient who appears septic. The Reynolds pentad adds mental status changes and sepsis to the triad. For example. The etiology is uncertain. a history of the following increases the risk of cholangitis: o Gallstones. chills. ulceration. Charcot described cholangitis as a triad of findings of right upper quadrant (RUQ) pain. Abdominal pain and jaundice is thought to occur in 70% and 60% of patients. combined with Charcot's triad. A history of abdominal pain or symptoms of gallbladder colic may be a clue to the diagnosis. y y y y y y y y Charcot's triad consists of fever. Consequently. fever. and jaundice. However. respectively.Clinical History In 1877. It is reported in up to 50-70% of patients with cholangitis. Physical . Fever is present in approximately 90% of cases. jaundiced. CBD stones o Recent cholecystectomy o Endoscopic manipulation or ERCP. These signs. elderly persons) are too ill to localize the source of infection. The management of this condition is described below. and obstruction. many patients with ascending cholangitis do not present with the classic signs and symptoms. Other symptoms include the following: o Jaundice o Fever. although decompression is usually not necessary. With septic shock. the diagnosis can be missed in up to 25% of patients. however. Patients present with altered mental status 10-20% of the time and hypotension approximately 30% of the time. recent studies believe it is more likely to be present in 15-20% of patients. and jaundice. A spectrum of cholangitis exists.4 Most patients complain of RUQ pain.

Most CBD stones are immediately symptomatic. followed by ERCP and tumors. o Pancreatic cancer 5 o Cholangiocarcinoma 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 . o Approximately 10-15% of patients with cholecystitis have CBD stones.y y In general. patients with cholangitis are quite ill and frequently present in septic shock without an apparent source of the infection. parasitic infection. choledocholithiasis is the most common cause of acute cholangitis. y y y CBD stones predispose patients to cholangitis. o Approximately 1% of patients post cholecystectomy have retained CBD stones. Partial obstruction is associated with an increased rate of infection compared with that of complete neoplastic obstruction. Partial obstruction is associated with a higher rate of infection than complete obstruction. Obstructive tumors cause cholangitis. 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. or extrinsic compression by the pancreas. can result in bacterial infection and cholangitis. Physical examination may reveal the following: o Fever (90%). Any condition that leads to stasis or obstruction of bile in the CBD. o Some CBD stones are formed primarily rather than secondarily to gallstones. including benign or malignant stricture. while some remain asymptomatic for years.

Bile is a watery liquid made by the cells of the liver that is important for digesting food in the intestine. progressive (worsening over time) disease of the bile ducts that channel bile from the liver into the intestines. . MD Medical Editor: Jay W.Font Size A A A y y y y y y y y 1 2 3 4 5 6 7 Next » y y y Primary Sclerosing Cholangitis Index Glossary Find a Local Doctor Primary Sclerosing Cholangitis Medical Author: Dennis Lee. Marks. one of them is manufacturing bile. particularly fat. The liver performs many functions. MD y y y y y y y y y 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).

itching. scarred and thickened (sclerotic). The bile then flows within the intrahepatic bile ducts out of the liver and into the extrahepatic bile ducts. Primary Sclerosing Cholangitis (cont.and ridding the body of toxins. Liver cells secrete the bile they make into small canals within the liver. the bile flows into the intestine where the bile mixes with food. jaundice. the intrahepatic and extrahepatic bile ducts become inflamed.) In this Article y y y y y y y y y y y 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 . From the extrahepatic bile ducts. infection in the bile ducts (cholangitis). The bile flows through the canals and into larger collecting canals (ducts) within the liver (the intrahepatic bile ducts). and finally obstructed. narrowed. Obstruction of the ducts can lead to abdominal pain. and liver scarring that leads to liver cirrhosis and liver failure. In primary sclerosing cholangitis.

this small subset of primary sclerosing cholangitis patients are believed to have an immune disorder causing their primary sclerosing cholangitis. fever. A small subset (approximately 10%) of primary sclerosing cholangitis patients has a rapidly progressive form of the disease with early onset of abdominal pain.000 people. There is a strong association between primary sclerosing cholangitis and chronic ulcerative colitis. fever. As primary sclerosing cholangitis progresses. The mean age at diagnosis of primary sclerosing cholangitis is around 40 years of age. Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus).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. The reason for the pruritus is not entirely known. What are the symptoms of primary sclerosing cholangitis? Most patients with early primary sclerosing cholangitis have no symptoms. fatigue. jaundice and fever than the majority of patients with the more indolent form of primary sclerosing cholangitis. patients may develop jaundice (yellowing of skin and darkening of urine). The patients with the autoimmune form of primary sclerosing cholangitis have more rapid and early onset of symptoms of abdominal pain. and itching that responds dramatically to treatment with corticosteroids. and systemic lupus erythematosus. 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. Since corticosteroids (such as prednisone) are medications for treating immune diseases such as ulcerative colitis. The accumulation of bilirubin turns the skin and whites of the eye (sclera) yellow. It is more common in men then in women. These patients also are at risk of developing primary sclerosing cholangitis complications. What causes primary sclerosing cholangitis? The cause of primary sclerosing cholangitis is not known. a disease of the intestines that is related to ulcerative colitis. As the disease progresses. Primary sclerosing cholangitis can also occur alone or in association with Crohn's disease. patients typically develop right upper abdominal pain. and jaundice. It may be due to accumulation of bile salts in the body. pruritus. approximately 70% of primary sclerosing cholangitis patients are men. Crohn's disease. 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. . also as a result of obstruction of the bile ducts.

Patients at highest risks for developing cholangiocarcinoma are primary sclerosing cholangitis patients with cirrhosis who also have long-standing ulcerative colitis. one of the more common reasons for liver transplantation. Cholangitis The narrowing of the bile ducts predisposes the bile to bacterial infection (cholangitis).Primary Sclerosing Cholangitis (cont. and mental confusion with progression to coma (hepatic encephalopathy). internal bleeding from rupture of esophageal varices. Colon cancer . Patients with advanced cirrhosis may develop frequent infections. jaundice. shaking chills (rigors). and upper abdominal pain. leading to the consideration of liver transplantation. the disease causes cirrhosis of the liver (irreversible scarring of the liver) and liver failure. Sepsis can cause damage to kidneys and lungs and even cause shock.) In this Article y y y y y y y y y y y 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. Cholangitis can result in bacterial infection spreading to the blood stream (a condition called sepsis). fluid in the ankles and the abdomen (ascites). Primary sclerosing cholangitis is. An estimated 9%-15% of patients with primary sclerosing cholangitis will develop cholangiocarcinoma. a very lethal type of cancer. Cholangiocarcinoma Cholangiocarcinoma (cancer of the bile ducts) is more common among patients with primary sclerosing cholangitis. in fact. Cholangitis is a serious and potentially life-threatening infection with fever.

gallstones in the bile ducts. itching. Radiologic tests Endoscopic retrograde cholangio-pancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) are commonly performed to visualize the intrahepatic and extrahepatic ducts. bile duct cancers and strictures. It is also important to exclude other diseases that can mimic primary sclerosing cholangitis. These ducts are typically normal in appearance in patients with PBC. Antimitochondrial antibody (AMA).Long-standing chronic ulcerative colitis alone is a risk factor for colon cancer. and jaundice) and abnormal laboratory tests (such as abnormally elevated blood levels of alkaline phosphatase and other liver enzymes). Except in those patients with the autoimmune form of primary sclerosing cholangitis. Primary Sclerosing Cholangitis (cont. and is confirmed by demonstration of abnormally thickened bile ducts using special radiological tests. These diseases include primary biliary cirrhosis (PBC).) In this Article y y y y y y y y y y y 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. Patients with both primary sclerosing cholangitis and ulcerative colitis have an even higher risk for developing colon cancer than patients with ulcerative colitis alone. which is elevated in patients with PBC. Blood tests The blood level of alkaline phosphates is usually elevated in primary sclerosing cholangitis. the bilirubin usually is normal but gradually increases as the disease progress. The blood levels of other liver enzymes (AST and ALT) may also be mildly elevated. is usually normal in patients with primary sclerosing cholangitis. but in primary .

sclerosing cholangitis patients.) 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: y y y y cholestyramine (Questran) or rifampin (Rifadin) to diminish itching Antibiotics for infection. Also. and. ERCP has the advantage of obtaining cell samples (a process called brush cytology) from the bile ducts. Colonoscopy Patients with primary sclerosing cholangitis have a high likelihood of also having ulcerative colitis. doctor can also insert balloons and stents across major areas of narrowing (dominant strictures) to relieve obstruction and treat infection. as mentioned previously. specifically cholangitis Vitamin D and calcium to prevent bone loss (osteoporosis) Medications such as ursodiol (UDCA) to retard the progression of primary sclerosing cholangitis . Brush cytology is not very accurate. colonoscopy is important to both diagnPrimary In this Article y y y y y y y y y y y Sclerosing Cholangitis (cont. patients with primary sclerosing cholangitis and ulcerative colitis have higher risks of developing colon cancer. However. Thus. MRCP is noninvasive and safe. but sometimes can help to diagnose cholangiocarcinoma. these ducts have a beaded appearance (multiple narrowings along the ducts with areas of widening in between). ERCP is more invasive and carries a 5%-6% chance of causing an attack of acute pancreatitis. during ERCP.

In selected primary sclerosing cholangitis patients with dominant strictures. Some doctors also believe that successful dilatation of dominant strictures decreases the risk of developing cholangitis. Except in the case of prednisone for autoimmune form of primary sclerosing cholangitis. at least one study showed that UDCA may decrease colon cancer risk in patients with both primary sclerosing cholangitis and chronic ulcerative colitis. Gengraf)] have been studied to treat primary sclerosing cholangitis. most doctors are treating primary sclerosing cholangitis patients with high dose UDCA (20-30 mg/kg/day) since UDCA is safe and well tolerated. ERCP and balloon dilatation (stretching) of the stricture can improve symptoms and abnormal blood levels of liver enzymes and bilirubin. UDCA is the most extensively studied medication for primary sclerosing cholangitis. doctors often also perform brush cytology of the dominant strictures to exclude cholangiocarcinoma. 6-mercaptopurine. ERCP and dilatation of dominant strictures should be done in centers with highly experienced physicians. However.y 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 y Medications Many medications [such as ursodiol (UDCA). it has been shown to improve fatigue. During ERCP. However. colchicine. tacrolimus (Prograf). none of the other medications have shown a consistent benefit on survival or decreased need for liver transplantation. and improve blood levels of liver enzymes in patients with primary sclerosing cholangitis. 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. Moreover. and it may improve bile duct disease and retard the development of liver cirrhosis. Doctors are currently awaiting the results of a multi-center. prednisone. decrease bile duct inflammation. there is still no conclusive evidence that UDCA actually prolongs life or decreases the need for liver transplantation in primary sclerosing cholangitis patients. Trexall). placebo controlled trial of high dose UDCA in primary sclerosing cholangitis. cyclosporine (Neoral. Meanwhile. At standard doses (15 mg/kg/day). . methotrexate (Rheumatrex. UDCA is believed to protect against damaging effects of other bile acids on the liver cells and also induce formation of antioxidants. and decrease liver scarring. In higher doses (20-30 mg/kg/day) UDCA has been shown to improve blood liver enzymes. UDCA Ursodiol (UDCA) is a bile acid that is given orally and replaces other bile acids in the body.

albumin. blood levels of bilirubin. Liver transplantation Even with modern management. and lower the risk of cholangiocarcinoma. Transplantation is now the definitive treatment in primary sclerosing cholangitis patients with advanced cirrhosis and liver failure. Font Size A A A y y y y y y « Previous 1 2 3 4 5 . most primary sclerosing cholangitis patients will die within 10 years of the time of diagnosis without liver transplantation. However. One year survival following transplantation is 85%-90%.Surgery is another treatment for dominant extrahepatic strictures in primary sclerosing cholangitis patients. The model suggests that when a patient's score estimates 6 month-survival is less than 80%. They are: y y y y 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. Reasons for liver transplant in primary sclerosing cholangitis patients are similar to those in other forms of end-stage liver disease. This model includes age. ose ulcerative colitis and for early detection of cancer or precancerous conditions. delay liver transplantation. and five year survival is as high as 85%. AST and a history of bleeding from esophageal varices. the patient should be considered for liver transplantation. 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. 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. In carefully selected patients.

Primary sclerosing cholangitis has a strong association with ulcerative colitis and cancer of the bile ducts. Primary sclerosing cholangitis is diagnosed on the basis of abnormal blood tests and radiologic y y y . progressive disease of the liver. The primary symptoms of primary sclerosing cholangitis are due to obstruction of the bile ducts and cirrhosis of the liver.y y y 6 7 Next » y y y Primary Sclerosing Cholangitis Index Glossary Find a Local Doctor Primary Sclerosing Cholangitis (cont. The cause of primary sclerosing cholangitis is not known though an immune cause is suspected in at least a minority of patients.) In this Article y y y y y y y y y y y 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 y y Primary sclerosing cholangitis is a chronic.

endoscopy. . y Primary sclerosing cholangitis is treated with medications. and liver transplantation.imaging of the bile ducts.

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