The Charcot’s triad of fever, jaundice and right upper quadrant abdominal pain is often times suggestive of cholangitis.

Systemic symptoms of fever is observable in 95% of all cases due to widespread inflammation and sepsis which are usually coupled with chills. Fatigue may also result in the ineffective breakdown of fat by the liver due to an impending obstruction in bile duct [7]. Generalized pruritus maybe observable due to the nerve irritation of the skin dermatomes by the accumulation of bile pigments in the system secondary to obstruction. These bile pigments may discolor the skin and the sclera of the eyes causing jaundice. Chronic cholangitis may show significant weight loss due to protein wasting by the liver.

An enlarged abdomen secondary to ascites or the accumulation of extracellular fluid in the abdominal cavity due to the increased intra hepatic pressure with portal hypertension. The elicit obstruction of the portal vein will increase the intrahepatic pressure causing the extracellular fluid to leak out of the liver. The intrahepatic pressure will cause varicosities in the esophagus which may prove to be fatal when it disrupts while external varicosities in the legs may also be seen outwardly.

• Splenomegaly

  Hepatomegaly, splenomegaly, jaundice, portal hypertension and, in more advanced cases, spider angiomata, ascites and muscle atrophy are observed. [orpha.net]

  […] include a general feeling of ill health (malaise); abdominal pain, especially the upper right portion of the abdomen; nausea; dark urine; light-colored stools; unintended weight loss, and/or abnormal enlargement of the liver (hepatomegaly) and/or spleen (splenomegaly [rarediseases.org]

• Pain

  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. [emedicine.medscape.com]

  Abdominal pain is the most common complaint and is seen in about 80% of patients with cholangitis. 8,10 However, patients, especially elderly patients, may present without abdominal pain. [emdocs.net]

  pain, Nausea, Vomiting Y’ I Y N CBD Roux-en-Y/CE - No recurrence at 2 mo Shanti 19 2001 33 B M Abdominal pain, jaundice N Y N IHBD (bilat) CE, hepatojejunostomy - No recurrence 3mo Shanti 19 2001 57 B F Abdominal pain, jaundice Y I Y N CBD Roux-en-Y [sciencerepository.org]

  The pain lasts longer than 6h and is usually associated with nausea/vomiting, fever and right upper quadrant pain. Choledocholithiasis – gallstones in the common bile duct. [sketchymedicine.com]

  An adverse event (catheter migration) occurred in 1 patient, and 17 patients developed pain after PC. During the same period, 104 patients underwent PC for cholecystitis. Adverse events occurred in 7 patients, and 62 developed pain. [ncbi.nlm.nih.gov]

• Fever

  Physical examination may reveal the following: Fever (90%), although elderly patients may have no fever RUQ tenderness (65%) Mild hepatomegaly Jaundice (60%) Mental status changes (10-20%) Sepsis Hypotension (30%) Tachycardia Peritonitis (uncommon, and [emedicine.medscape.com]

  Systemic Inflammation Fever or Shaking Chills Body Temperature >38°C Laboratory evidence of inflammatory response WBC <4,000 or >10,000 CRP >1 B. [emdocs.net]

  A biliary colic will present with a colicky RUQ pain yet without fever, leucocytosis, or jaundice. Cholecystitis will present with RUQ pain and fever yet jaundice will be absent. [teachmesurgery.com]

  Some PSCers say it feels a bit like having flu and symptoms can include: itching (pruritus) dark urine temperature shivers and chills* fever* abdominal pain in the Right Upper Quadrant (RUQ) jaundice pale stools nausea/vomiting night sweats *Fever, shivers [pscsupport.org.uk]

  Clinical dx of cholangitis: Charcot's triad: fever, RUQ pain and jaundice Reynold's pentad: as above + hypotension and AMS a retrospective review of 108 pts with a diagnosis of acute cholangitis found that 42% of pts had Charcot's triad and 3% had Renolyd's [errolozdalga.com]

• Malaise

  In early stages, unspecific manifestations such as fever, fatigue, pruritus, malaise, abdominal pain, and weight loss are reported, however about 40-50% of patients may be asymptomatic at time of diagnosis. [orpha.net]

  […] bilioenteric anastomosis) Predisposing factors gallstones previous cholecystectomy ERCP or cholangiogram previous cholangitis immunocompromise malignancy CLINICAL FEATURES History fever (>80%) abdominal pain (especially RUQ) (>80%) nausea and vomiting, malaise [lifeinthefastlane.com]

  Fever with/without chills Nausea and/or vomiting Yellow skin – obstructive jaundice Dark urine Pale stools or clay colored stools Itchy skin – pruritis Malaise It is not uncommon for patients to report repeated episodes of biliary colic prior to the onset [healthhype.com]

• Rigor

  Presentation – rigors, fever, abdominal pain, jaundice Treatment It is a medical emergency. [almostadoctor.co.uk]

  This physiology generates characteristic bacteremia and rigors. Ascending cholangitis has a greater tendency to evolve rapidly into septic shock. [emcrit.org]

  On examination, patients may have pyrexia (in 90% of cases), rigors, jaundice, right upper quadrant tenderness, confusion, hypotension, and tachycardia may be present. [teachmesurgery.com]

  Other symptoms include the following: Jaundice Fever, chills, and rigors Abdominal pain Pruritus Acholic or hypocholic stools Malaise The patient's medical history may be helpful. [emedicine.medscape.com]

  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 ). [emedicinehealth.com]

• Lymphadenopathy

  An abdominal ultrasound and a computed tomography scan were performed, revealing a gigantic lymphadenopathy mass compressing the common bile duct and the duodenum. [ncbi.nlm.nih.gov]

  Other MRI findings include periportal lymphadenopathy, periportal high T2 signal, and abnormal hyperintensity of the liver parenchyma (6). [sonoworld.com]

  Pathology of supraclavicular lymphadenopathy in Chandigarh, north India: an audit of 200 cases diagnosed by needle aspiration. Cytopathology 17, 94–96 (2006). 89 Taylor, K. J. W., Rosenfield, A. T. & Spiro, H. M. [nature.com]

  Porta hepatis lymphadenopathy from lymphomas or metastatic tumors can cause extrahepatic biliary obstruction. Mirizzi’s syndrome can present with cholangitis when an impacted gallbladder stone causes common hepatic duct obstruction. [clinicaladvisor.com]

• Abdominal Pain

  Abdominal pain is the most common complaint and is seen in about 80% of patients with cholangitis. 8,10 However, patients, especially elderly patients, may present without abdominal pain. [emdocs.net]

  No recurrence 6mo Vauthey 2 2003 44 - M Abdominal pain, jaundice Y NMCP Y Y CBD (distal) - CS No recurrence 18mo Jimenez-Saenz 20 2003 67 - F Abdominal pain, jaundice Y E Y Y IHBD/EHBD† CE CS No recurrence 12mo Duseja 21 2005 16 - F Abdominal Pain, [sciencerepository.org]

  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. [emedicine.medscape.com]

  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. [emedicinehealth.com]

• Right Upper Quadrant Pain

  It is estimated that 50% to 70% of these patients present with right upper quadrant pain, fever, and jaundice. [bestpractice.bmj.com]

  Clinical findings Right upper quadrant pain that may radiate to the shoulder, variable severity, nausea, vomiting, constipation, episodic chills, fever, slow pulse, Murphy sign, anorexia, weight loss; spontaneous remission is rare. [medical-dictionary.thefreedictionary.com]

  The pain lasts longer than 6h and is usually associated with nausea/vomiting, fever and right upper quadrant pain. Choledocholithiasis – gallstones in the common bile duct. [sketchymedicine.com]

  Snap Shot A 50-year-old woman presents with fever, jaundice, and right upper quadrant pain. Labs reveal an elevated direct bilirubin and alkaline phosphatase. [step2.medbullets.com]

• Upper Abdominal Pain

  As primary sclerosing cholangitis progresses, individuals typically develop right upper abdominal pain, fever, fatigue, pruritus, and jaundice. These individuals also are at risk of developing primary sclerosing cholangitis complications. [emedicinehealth.com]

  The common signs and symptoms associated with Cholangitis include: Fever Jaundice Right upper abdominal pain Nausea and vomiting The first 3 symptoms are collectively called Charcot’s triad. [dovemed.com]

  Ninety percent of the patients complain of upper abdominal pain and have enlarged intra- and extrahepatic bile ducts on abdominal ultrasonography. [dx.doi.org]

• Acute Abdomen

  The acute abdomen during pregnancy. Clin Obstet Gynecol. 2002; 45 :405–13. doi: 10.1097/00003081-200206000-00011. [ PubMed ] [ CrossRef ] [ Google Scholar ] 61. Barone JE, Bears S, Chen S, Tsai J, Russell JC. [ncbi.nlm.nih.gov]

  Sharp, HT 2002 The acute abdomen during pregnancy Clin Obstet Gynecol 45 405 13 CrossRef PubMed Google Scholar 61. [dx.doi.org]

  Tenderness in the right upper abdomen, a palpable gallbladder, and Murphy’s sign are the characteristic findings of acute cholecystitis. A positive Murphy’s sign shows 79–96 % specificity [ 18, 20 ] for acute cholecystitis. [link.springer.com]

• Hypotension

  Clinical dx of cholangitis: Charcot's triad: fever, RUQ pain and jaundice Reynold's pentad: as above + hypotension and AMS a retrospective review of 108 pts with a diagnosis of acute cholangitis found that 42% of pts had Charcot's triad and 3% had Renolyd's [errolozdalga.com]

  Factors which increase the mortality rate include delayed diagnosis, liver failure, cirrhosis, CKD, hypotension, female gender, and >50yrs. [teachmesurgery.com]

  Charcot’s triad includes right upper quadrant abdominal pain, fever, and jaundice, while the addition of mental status change and hypotension constitutes Reynold’s pentad. [emdocs.net]

  Occurs in <5% Hypotension may be the only presenting sign in elderly patients Differential Diagnosis RUQ Pain Gallbladder disease Acute cholecystitis Cholangitis Symptomatic cholelithiasis Choledocholithiasis Acalculous cholecystitis Peptic ulcer disease [wikem.org]

  Acute suppurative cholangitis occurs with the presence of pus in the biliary ducts and may result in Reynold’s pentad: Charcot’s triad plus hypotension and confusion. If present, the disease can become rapidly fatal. [medcomic.com]

• Flushing

  Normally bacteria from the gut has a pretty tough time ascending up the bile ducts, and this is because bile flows down from the gallbladder, along with some pancreatic juice from the pancreas, into the duodenum, and this tends to flush out any bacteria [osmosis.org]

  […] due to increased intra-biliary pressures as a result of obstruction translocation of pathogenic micro-organisms from the duodenum up the biliary tree and into the bloodstream infection overcomes normal barriers: mucosal barriers, continuous biliary flushing [lifeinthefastlane.com]

  Feb 27, The amount of time and effort it takes to flush weed out of your urine Here are several ways to flush marijuana out of your body naturally. 1. [egobogapis.tk]

• Hyperpigmentation

  […] due to liver failure (ascites) Fatty deposits (xanthomas) on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees Yellowing of the skin and eyes (jaundice) Darkening of the skin that's not related to sun exposure (hyperpigmentation [mayoclinic.org]

  […] symptoms of chronic cholangitis may include: tiredness and fatigue itchy skin dry eyes dry mouth If you have chronic cholangitis for a long time, you may have: pain in the upper right side night sweats swollen feet and ankles darkening of the skin (hyperpigmentation [healthline.com]

• Kidney Failure

  Whereas less severe cases with appropriate treatment have very good prognosis, severe cases with delayed treatment may result in grave complications such as sepsis or kidney failure Addressing issues of biliary tract obstruction in a timely manner may [dovemed.com]

  […] injury and the presence of liver abscesses.[23] Complications following severe cholangitis include kidney failure, respiratory failure (inability of the respiratory system to oxygenate blood and/or eliminate carbon dioxide), abnormal heart rhythms, wound [en.wikipedia.org]

A magnetic resonance imaging of the bile duct nomenclature including the liver, the gallbladder and the pancreas may demonstrate the inherent inflammation of the bile duct walls. Radiographic elucidation using radioactive dyes to demonstrate the biliary tree via X-ray may also do the trick [5].

A percutaneous liver biopsy or an insertion of a needle in the chest cavity to get a sample of liver tissue may microscopically demonstrate the impending liver failure and herald the attending doctors on how aggressive they should proceed with the inflammation.

Laboratory studies on liver functions may show elevated levels of liver enzymes may be suggestive of an inflammatory process involving the liver and its neighboring structure.

• Pseudomonas

  The most common pathogens isolated in blood cultures are E coli (59%), Klebsiella species (16%), Pseudomonas aeruginosa (5%), and Enterococcus species (4%). [emedicine.medscape.com]

  PSC) Elevated intraluminal pressure in the gallbladder leads to translocation of bacteria Bacteria may gain access via lymphatics, portal venous blood or retrograde from the duodenum Common pathogens: E. coli, Klebsiella, Streptococcus, Enterobacter, Pseudomonas [coreem.net]

• Gram-Positive Bacteria

  Common infecting organisms include gram-negative bacteria (eg, Escherichia coli, Klebsiella sp, Enterobacter sp); less common are gram-positive bacteria (eg, Enterococcus sp) and mixed anaerobes (eg, Bacteroides sp, Clostridia sp). [msdmanuals.com]

  Common infecting organisms include gram-negative bacteria (eg, Escherichia coli, Klebsiella species, Enterobacter species); less common are gram-positive bacteria (eg, Enterococcus species) and mixed anaerobes (eg, Bacteroides species, Clostridia species [merckmanuals.com]

  Vancomycin is recommended when patients are colonized with resistant Gram‐positive bacteria such as methicillin‐resistant Staphylococcus aureus and/or Enterococcus spp. or these multidrug‐resistant Gram‐positives are of concern. [onlinelibrary.wiley.com]

  Tigecycline has in-vitro activity against a wide range of clinically significant Gram-positive and Gram-negative bacteria [ 41 ]. [link.springer.com]

• Hepatic Necrosis

  Leukocytosis is common, and aminotransferases may reach 1000 IU/L, suggesting acute hepatic necrosis, often due to microabscesses. Blood cultures guide antibiotic choice. [msdmanuals.com]

  The other two patients, despite the initial decline in bleeding, developed massive hepatic necrosis over the next week, followed by multiorgan failure and death. [doi.org]

Medical Approach

The treatment of acute and non-progressive cholangitis focuses on monitoring and symptomatic relief. The generalized pruritus is simply treated with antihistamines like Cetirizine, Loratidine and Diphenhydramine [1]. Bile acid binding agents may alleviate cholestasis and reduce itching and jaundice. Parenteral antibiotics to combat the specific pathogen in cholangitis may control the spread of infection and avert generalized sepsis. The inability of the liver to process certain vitamins like calcium and vitamin D may require parenteral infusion to protect further complications with the bones.

Surgical Approach

The surgical option focuses on the relief of the obstruction of the bile duct when it is identified as the cause of the cholangitis. Stenting of the bile ducts may relieve the ducts of obstructing stones, tumorous blockages may be surgically resected to relieve the bile flow and balloon dilatation of the bile duct using endoscopy may stretch the bile ductwalls to correct anatomical obstruction of the duct caliber. Liver transplant are reserved for those who progress from Primary Sclerosing Cholangitis to irreparable liver damage [10].

The medical treatment of acute cholangitis directed to the exact pathogens may eradicate the infections in some cases. Untreated infections may lead to septic shock and death. Cholangitis due to obstructive tumors and stones is corrected with the restoration of the bile flow via surgery and may offer promising results and outcome.

Primary sclerosing cholangitis may chronically lead to cirrhosis and liver failure may warrant a liver transplant as a definitive course of action [3]. Those patients with comorbid afflictions like inflammatory bowel diseases and HIV-AIDS may have poorer prognosis than the former.

Complications

The following medical conditions are the leading complications of cholangitis:

• Bile duct cancer may develop with the chronic and progressive scarring of the bile duct walls and its contiguous organs [8].
• Colon carcinoma is a very common complication of primary sclerosing cholangitis especially with comorbid inflammatory bowel diseases like ulcerative colitis and Crohn disease [9].
• Liver failure will result from the progressive scarring of the liver or cirrhosis as a result of the chronic infection of the bile ducts.
• Portal hypertension will develop when the portal vein from the mesenteric vessels to the liver scars and increases the pressure towards the liver.

Acute cholangitis may result from the spreading infection from proximal organs like the liver, the duodenum and the pancreas often times due to gram negative bacteria from the intestinal flora. Infection of the bile ducts often precedes a blockage of the bile flow by a tumor, round worm bolus or gallstone.

The origins of Primary Sclerosing Cholangitis however remains elusive to but theories point to immune response, toxins and infection. Patients with compromised immune response like HIV patients and those with Inflammatory Bowel Diseases are more prone to cholangitis [6].

Cholangitis may occur at any age but is seen more frequently within the age group of 25 to 45 years old. Men are more affected than their women counterparts. A great majority of patients with Ulcerative Colitis or Crohn Disease has or may develop cholangitis.

The infection in cholangitis may be primary or acquired in nature. Primary Sclerosing Cholangitis presents as the progressive thickening of the bile duct walls during inflammation which may be triggered by an immune response to toxins or infections. Acquired or secondary cholangitis is more commonly caused by choledocholithiasis or lodged bile stones in the bile ducts [2].

Cholangitis may also be frequent in iatrogenic manipulation of the bile duct anatomy during hepato-biliary surgery during the placement of bile stone stents. Less common causes like tumors and carcinomas may give rise to the inflammation process as well.

Opportunistic bacterial infection of the bile ducts are documented in a number of cases of HIV-AIDS patients and severely immune compromised patients [4].

Patients should adequately be immunized with hepatitis A and hepatitis B to prevent chronic liver damaging complications. Alcohol should be taken in moderation to prevent liver damage. Body weight should be maintained at optimal levels along with a healthy diet to prevent gallstones.

Patients diagnosed with gallstones should have it removed when first discovered to prevent inflammatory complications. Stenting the bile duct in cases of choledocholithiasis cases can avert this event. Tumors and cancerous growth discovered radiographically should vie for surgical resection early. Anthelmintic drugs can prevent hookworm and round worm infestation that may also cause bile duct obstruction.

The liver delivers potent enzymes that aids in the digestion of fats and the neutralization of bodily toxins by producing bile. This potent yellow-green material is delivered through a network of tube conduits leading to the gastrointestinal tract.

Acute and chronic inflammation of these bile ducts is generally referred to as cholangitis. The progressive thickening of the walls due to chronic inflammation which eventually results to liver damage is more commonly known as primary sclerosing cholangitis.

Patients should be extra conscious when taking non-prescription medications. One should ask the pharmacist for the right dose to avoid liver damage. Patient should always inform their doctors or the pharmacists of their ongoing medications to avoid drug interactions that may inadvertently damage the liver.

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3. Lee KF, Chong CN, Ng D, et al. Outcome of surgical treatment for recurrent pyogenic cholangitis: a single-centre study. HPB (Oxford). 2009;11(1):75-80
4. Kadakia SC. Biliary tract emergencies. Acute cholecystitis, acute cholangitis, and acute pancreatitis. Med Clin North Am. Sep 1993;77(5):1015-36. 
5. Lameris JS, Overhagen HV. Imaging and intervention in patients with acute right upper quadrant disease. In: Bailliere's Clinical Gastroenterology. Vol 9. Harcourt Brace & Co;1995:21-36.
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7. Lipsett PA, Pitt HA. Acute cholangitis. Surg Clin North Am. Dec 1990;70(6):1297-312. 
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9. Lee JG. Diagnosis and management of acute cholangitis. Nat Rev Gastroenterol Hepatol.
10. Shojaiefard A, Esmaeilzadeh M, Ghafouri A, Mehrabi A. Various techniques for the surgical treatment of common bile duct stones: a meta review. Gastroenterol Res Pract. 2009;2009:840208.