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Cholangitis

PSC


Presentation

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.

Regurgitation
  • 2-4 An increased intrabiliary pressure, therefore, has been suspected to play a significant role in the pathogenesis of septicemia in cholangitis. 2,4,5 In order to determine the significance of biliary pressure in initiating the process of biliary regurgitation[oadoi.org]
Hepatic Mass
  • Among the eight cases with diagnoses reported, six patients were misdiagnosed as intrahepatic cholangiocarcinoma; one was diagnosed as hepatic mass and one as IgG4-SC before biopsy or operation.[ncbi.nlm.nih.gov]
  • Ling M, Begun J, Patil S, Hamilos DL (2011) Eosinophilic Cholangiopathy Presenting with Peripheral Eosinophilia and Hepatic Mass. J Allergy Clin Immun 127: 108-108. 30.[sciencerepository.org]
Hyperkeratosis
  • Clinical examination revealed jaundice, poikiloderma, diffuse cigarette paper-like atrophy on dorsal surfaces of the hands, and palmoplantar hyperkeratosis. Skin biopsy showed epidermal atrophy covered by orthokeratotic hyperkeratosis.[ncbi.nlm.nih.gov]
Photosensitivity
  • He presented with neonatal blistering and developed photosensitivity and changes in skin pigmentation during childhood.[ncbi.nlm.nih.gov]

Workup

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.

Listeria Monocytogenes
  • Listeria monocytogenes is a well-known cause of meningitis in immunocompromised patients. This organism has a growing significance for community-acquired meningitis, which should have ampicillin added to the usual regimen.[ncbi.nlm.nih.gov]

Treatment

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].

Prognosis

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.

Etiology

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].

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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].

Prevention

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.

Summary

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.

Patient Information

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.

References

Article

  1. Li FY, Cheng NS, Mao H, Jiang LS, et al. Significance of controlling chronic proliferative cholangitis in the treatment of hepatolithiasis. World J Surg. Jul 30 2009;epub ahead of print
  2. Hanau LH, Steigbigel NH. Acute (ascending) cholangitis. Infect Dis Clin North Am. Sep 2000;14(3):521-46.
  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.
  6. Leung JW, Yu AS. Hepatolithiasis and biliary parasites. Bailliere's Clinical Gastroenterology. 1997;11:681-706.
  7. Lipsett PA, Pitt HA. Acute cholangitis. Surg Clin North Am. Dec 1990;70(6):1297-312. 
  8. Raraty MG, Finch M, Neoptolemos JP. Acute cholangitis and pancreatitis secondary to common duct stones: management update. World J Surg. Nov 1998;22(11):1155-61. 
  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.

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Last updated: 2019-07-11 22:29