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.

• 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]

• Fatigue

  Early symptoms of primary sclerosing cholangitis include fatigue and bodily itching (pruritus). As the disease progresses, individuals may develop jaundice (yellowing of skin and darkening of urine). [emedicinehealth.com]

  Symptoms usually begin gradually with worsening fatigue and itchiness. Jaundice (yellowish discoloration of the skin and whites of the eyes) tends to develop later. [merckmanuals.com]

  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]

  Treatment for symptoms such as fatigue or complications of the PSC (such as demineralization of the bones, osteoporosis) must be decided upon on a case-by-case basis. [drfalkpharma.de]

• Weight Loss

  A 25 years old lady, 34 weeks primigravida was referred from the Emergency Department to the Medical Unit Khyber Teaching Hospital-MTI, Peshawar with four weeks of fever, progressive jaundice, pruritus, night sweats and weight loss. [ncbi.nlm.nih.gov]

  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]

  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]

  Disease progression can cause chronic fatigue, loss of appetite, weight loss and jaundice. Long-term liver cell damage can lead to cirrhosis, a hardening of the liver that prevents it from functioning properly. [livestrong.com]

• Asymptomatic

  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]

  Most CBD stones are immediately symptomatic, while some remain asymptomatic for years. Some CBD stones are formed primarily rather than secondarily to gallstones. Obstructive tumors Obstructive tumors cause cholangitis. [emedicine.medscape.com]

  The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. [wwe.eurekamag.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]

• Nausea

  It may be missed for prolonged periods until it leads to symptoms like stone formation, abdominal pain, obstructive jaundice, nausea or vomiting or change sin stool color (pale or clay colored). [healthhype.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]

  Klebsiella, Streptococcus, Enterobacter, Pseudomonas Other causes: HIV/AIDS cholangiopathy, parasitic infections (Ascaris lumbricoides) Presentation Charcot’s Triad: Fever, RUQ pain and jaundice (neither sensitive nor specific) Symptoms Fever/chills Nausea [coreem.net]

• Vomiting

  It may be missed for prolonged periods until it leads to symptoms like stone formation, abdominal pain, obstructive jaundice, nausea or vomiting or change sin stool color (pale or clay colored). [healthhype.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]

  This can lead to abdominal pain, vomiting, fever, and other symptoms. Without treatment, the infection can spread to the liver and may become life threatening. [connecticutchildrens.org]

• 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]

• Pruritus

  Medical management of pruritus is directed by the severity of the underlying pruritus. Mild pruritus may be treated with skin emollients and possibly antihistamines. [gi.org]

  Pruritus was the most common adverse event; 15% (OCA 10 mg) and 38% (OCA 50 mg) discontinued due to pruritus. [ncbi.nlm.nih.gov]

  The Cleveland Clinic has published an excellent article entitled "Pruritus" which describes the various types and causes of itching. A comprehensive article on pruritus is as follows: "Pruritus" (American Family Physician). [psc-literature.org]

  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. [emedicinehealth.com]

• 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]

• Xanthelasma

  Xanthomata, particularly around the eyes (xanthelasma), are commonly found in patients with PBC. PBC is also associated with metabolic bone disease, resulting in premature osteoporosis. [clevelandclinicmeded.com]

• Palmar Erythema

  Presentations of primary sclerosing cholangitis History: Fatigue Pruritus Weight loss Right upper quadrant (RUQ) pain Recurrent biliary infections Examination: Jaundice Hepatomegaly Features of chronic liver disease Dupuytren’s contracture; palmar erythema [oxfordmedicaleducation.com]

• Dark Urine

  Dark urine and clay-colored stools. Nausea and vomiting. Yellowing of the skin (jaundice), which may come and go. Quick diagnosis and treatment are very important. Antibiotics to cure infection are the first treatment done in most cases. [nlm.nih.gov]

  Obstruction of the biliary system can hinder excretion of bilirubin into the gut and simultaneously exceed the processing ability of the kidneys, leading to pale stools and dark urine. [accessmedicine.mhmedical.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]

  Symptoms Symptoms may include: abdominal pain in the upper right area of the gut fever and chills clay-colored stools dark urine nausea and vomiting jaundice (a yellow tinge that appears in the eyes and on the skin) Pain can be intermittent, and may also [muschealth.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]

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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