Cholangitis

Updated: Mar 11, 2020
  • Author: Homayoun Shojamanesh, MD; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Overview

Background

Cholangitis is an infection of the biliary tract with the potential to cause significant morbidity and mortality. Many patients with acute cholangitis respond to antibiotic therapy; however, patients with severe or toxic cholangitis may not respond and may require emergency biliary drainage. Jean M. Charcot recognized this illness in 1877 when he described a triad of fever, jaundice, and right upper quadrant pain. [1] In 1959, Reynolds and Dargon described a more severe form of the illness that included the additional components of septic shock and mental confusion, which is referred to as the Reynolds pentad. [1]

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Pathophysiology

Historically, choledocholithiasis was the most common cause of biliary tract obstruction resulting in cholangitis. Over the past 20 years, biliary tract manipulations/interventions and stents have reportedly become more common causes of cholangitis. Hepatobiliary malignancies are a less common cause of biliary tract obstruction and subsequent bile contamination resulting in cholangitis. [2]

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Etiology

The two main causes of cholangitis are common bile duct stones and biliary tract manipulation. [3] In patients with a stent in situ, cholangitis indicates stent block and need for change. Other possible causes of biliary tract obstruction that may lead to infection include strictures, tumors, choledochal/biliary cysts, or sump syndrome. Hepatolithiasis is also a possible cause of cholangitis [4] and is observed more frequently in East Asia. More than 90% of patients with hepatolithiasis have calcium bilirubinate stones, also referred to as brown pigment stones. Worms (eg Ascaris, Clonorchis) in the biliary tract can also cause cholangitis. Cholangitis in patients with a biliary-enteric anastomosis (eg, hepatico-jejunostomy or a bilio-biliary anastomosis) after liver transplant indicates anastomotic stricture.

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Epidemiology

Race-, sex-, and age-related demographics

Cholangitis is reported in all races. One variant, Asian cholangitis (also referred to as Oriental cholangio-hepatitis [OCH] or recurrent pyogenic cholangitis [RPC]), is observed with increased frequency in Southeast Asia. [5]

The condition is reported in both females and males and has no clear predominance in either.

It mostly occurs in adults, with a reported median age at onset of 50-60 years. In neonates, extrahepatic biliary atresia (EHBA) is a cause of cholangitis. In children and young adults, choledochal cyst can cause cholangitis.

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Prognosis

The prognosis is usually guarded, although it improves with early antibiotic treatment and appropriate drainage and decompression of biliary tract as needed. Factors reportedly associated with a poor prognosis include old age, female sex, acute renal failure, preexisting cirrhosis, and malignant biliary obstruction.

Mortality/morbidity

The mortality rate of acute cholangitis ranges from 5-10%, with a higher mortality rate in patients who require emergency biliary decompression or surgery.

Cholangitis has significant potential for mortality and morbidity, especially if left untreated. Reported mortality rates have been as high as 88% for untreated cholangitis.

Complications

Complications include pyogenic liver abscess, cholangiolytic abscess (usually small and multiple) in the liver, longstanding recurrent cholangitis (eg, Asiatic cholangitis), and acute renal failure. Longstanding recurrent cholangitis can cause secondary biliary cirrhosis (SBC), portal hypertension, and liver failure. [6]

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