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A person with cholangitis may complain of [[abdominal pain]] (particularly in the [[right upper quadrant (abdomen)|right upper quadrant]] of the [[Human abdomen|abdomen]]), [[fever]], [[rigor (medicine)|rigors]] (uncontrollable shaking) and a feeling of uneasiness ([[malaise]]). Some may report [[jaundice]] (yellow discoloration of the skin and the whites of the eyes).<ref name=Kinney>{{cite journal | author=Kinney TP | title=Management of ascending cholangitis | journal=Gastrointest Endosc Clin N Am |date=April 2007 | volume=17 | issue=2 | pages=289–306 | pmid=17556149 | doi=10.1016/j.giec.2007.03.006}}</ref>
[[Physical examination]] findings typically include jaundice and right upper quadrant tenderness.<ref name=Kinney/> [[Charcot's cholangitis triad|Charcot's triad]] is a set of three common findings in cholangitis: abdominal pain, jaundice, and fever.<ref name=Charcot>{{cite book | author=Charcot JM | title=Leçons sur les maladies du foie, des voies biliaires et des reins faites à la Faculté de médecine de Paris: Recueillies et publiées par Bourneville et Sevestre | ___location=Paris | publisher=Bureaux du Progrés Médical & Adrien Delahaye |
In the elderly, the presentation may be atypical; they may directly collapse due to sepsis without first showing typical features.<ref name=schwartz/> Those with an indwelling stent in the bile duct ([[#Causes|see below]]) may not develop jaundice.<ref name=schwartz/>
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The biliary tree is normally relatively free of bacteria because of certain protective mechanisms. The sphincter of Oddi acts as a mechanical barrier. The biliary system normally has low pressure (8 to 12 [[centimetre of water|cmH<sub>2</sub>O]])<ref>{{cite book | author=Dooley JS | title=Oxford textbook of clinical hepatology | publisher=Oxford University Press | year=1999 | isbn=978-0-19-262515-1 | page=1650 }}</ref> and allows bile to flow freely through. The continuous forward flow of the bile in the duct flushes bacteria, if present, into the duodenum, and does not allow the establishment of an infection. The constitution of bile—[[bile salt]]s<ref name=Kinney/> and [[immunoglobulin]]<ref name=schwartz/> secreted by the [[epithelium]] of the bile duct also has a protective role.
Bacterial contamination alone in absence of obstruction does not usually result in cholangitis.<ref name=schwartz/> However increased pressure within the biliary system (above 20 cmH<sub>2</sub>O)<ref name=Huang>{{cite journal |vauthors=Huang T, Bass JA, Williams RD |title=The significance of biliary pressure in cholangitis |journal=Arch Surg |volume=98 |issue=5 |pages=629–632 |date=May 1969 |pmid=4888283 |doi=10.1001/archsurg.1969.01340110121014}}</ref> resulting from obstruction in the bile duct widens spaces between the cells lining the duct, bringing bacterially contaminated bile in contact with the blood stream. It also adversely affects the function of [[Kupffer cells]], which are specialized [[macrophage]] cells that assist in preventing bacteria from entering the biliary system. Finally, increased biliary pressure decreases production of [[IgA]] immunoglobulins in the bile.<ref name=Sung2>{{cite journal |vauthors=Sung JY, Costerton JW, Shaffer EA |title=Defense system in the biliary tract against bacterial infection |journal=Dig Dis Sci |volume=37 |issue=5 |pages=689–96 |date=May 1992 |pmid=1563308 |doi= 10.1007/BF01296423|s2cid=21258760
In ascending cholangitis, it is assumed that organisms migrate backwards up the bile duct as a result of partial obstruction and decreased function of the sphincter of Oddi.<ref name=Kinney/> Other theories about the origin of the bacteria, such as through the [[portal vein]] or transmigration from the [[colon (anatomy)|colon]], are considered less likely.<ref name=Kinney/>
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The definitive treatment for cholangitis is relief of the underlying biliary obstruction.<ref name=Kinney/> This is usually deferred until 24–48 hours after admission, when the patient is stable and has shown some improvement with antibiotics, but may need to happen as an emergency in case of ongoing deterioration despite adequate treatment,<ref name=Kinney/> or if antibiotics are not effective in reducing the signs of infection (which happens in 15% of cases).<ref name=schwartz/><ref name=Williams/>
[[Endoscopic retrograde cholangiopancreatography]] (ERCP) is the most common approach in unblocking the bile duct. This involves endoscopy (passing a [[fiberoptic]] tube through the stomach into the duodenum), identification of the ampulla of Vater and insertion of a small tube into the bile duct. A sphincterotomy (making a cut in the sphincter of Oddi) is typically done to ease the flow of bile from the duct and to allow insertion of instruments to extract gallstones that are obstructing the [[common bile duct]]; alternatively or additionally, the common bile duct orifice can be dilated with a balloon.<ref name=Heo>{{cite journal |vauthors=Heo JH, Kang DH, Jung HJ |title=Endoscopic sphincterotomy plus large-balloon dilation versus endoscopic sphincterotomy for removal of bile-duct stones |journal=Gastrointest Endosc |volume=66 |issue=4 |pages=720–6; quiz 768, 771 |date=October 2007 |pmid=17905013 |doi=10.1016/j.gie.2007.02.033|display-authors=etal}}</ref> Stones may be removed either by direct suction or by using various instruments, including balloons and baskets to trawl the bile duct in order to pull stones into the duodenum. Obstructions that are caused by larger stones may require the use of an instrument known as a mechanical [[lithotriptor]] in order to crush the stone prior to removal.<ref name=Caddy>{{cite journal |vauthors=Caddy GR, Tham TC |title=Gallstone disease: Symptoms, diagnosis and endoscopic management of common bile duct stones |journal=Best Pract Res Clin Gastroenterol |volume=20 |issue=6 |pages=1085–101 |year=2006 |pmid=17127190 |doi=10.1016/j.bpg.2006.03.002}}</ref> Obstructing stones that are too large to be removed or broken mechanically by ERCP may be managed by [[extracorporeal shock wave lithotripsy]]. This technique uses acoustic shock waves administered outside the body to break down the stones.<ref name=Hochberger>{{cite journal |vauthors=Hochberger J, Tex S, Maiss J, Hahn EG |title=Management of difficult common bile duct stones |journal=Gastrointest Endosc Clin N Am |volume=13 |issue=4 |pages=623–34 |date=October 2003 |pmid=14986790 |doi= 10.1016/S1052-5157(03)00102-8
Narrowed areas may be bridged by a [[stent]], a hollow tube that keeps the duct open. Removable plastic stents are used in uncomplicated gallstone disease, while permanent [[Self-expandable metallic stent|self-expanding metal stents]] with a longer lifespan are used if the obstruction is due to pressure from a tumor such as [[pancreatic cancer]]. A nasobiliary drain may be left behind; this is a plastic tube that passes from the bile duct through the stomach and the nose and allows continuous drainage of bile into a receptible. It is similar to a [[nasogastric tube]], but passes into the common bile duct directly, and allows for serial x-ray cholangiograms to be done to identify the improvement of the obstruction. The decision on which of the aforementioned treatments to apply is generally based on the severity of the obstruction, findings on other imaging studies, and whether the patient has improved with antibiotic treatment.<ref name=Kinney/> Certain treatments may be unsafe if [[coagulation|blood clotting]] is impaired, as the risk of bleeding (especially from sphincterotomy) is increased in the use of medication such as [[clopidogrel]] (which inhibits [[platelet]] aggregation) or if the [[prothrombin time]] is significantly prolonged. For a prolonged prothrombin time, [[vitamin K]] or [[fresh frozen plasma]] may be administered to reduce bleeding risk.<ref name=Kinney/>
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