Overall Bottom Line
- Acute cholecystitis and cholangitis are potential infectious complications of cholelithiasis (gallstones). Antibiotics covering bowel flora such as Enterobacteriaceae should be promptly initiated while considering surgical or radiologic intervention.
- Charcot’s triad of fever, jaundice and abdominal pain remains the clinical standard for the diagnosis of acute cholangitis.
- AIDS cholangiopathy refers to the rare syndrome of biliary strictures and obstruction associated with infections in patients with CD4 counts of <100/mm3.
- Endoscopic or radiologic treatment of the biliary strictures and highly active antiretroviral therapy play important roles in the treatment of AIDS cholangiopathy.
Section 1: Background
Definition of disease
- Biliary infections encompass a variety of clinical syndromes including acute cholecystitis, cholangitis and AIDS cholangiopathy.
- Cholecystitis is inflammation of the gall bladder and can further be defined as calculous, in which gallstones obstruct the cystic duct leading to distension and inflammation, or acalculous.
- Cholangitis is defined as bacterial infection in the setting of obstruction of the common bile duct and is usually secondary to choledocholithiasis.
- AIDS cholangiopathy is a rare condition described in patients with AIDS characterized by abnormalities in the bile ducts and often associated with parasitic infections or CMV.
Disease classification
- Acute cholecystitis is associated with the relatively abrupt onset of fevers and RUQ pain. Leukocytosis is common, but jaundice is rare.
- Chronic cholecystitis is classically a histopathologic diagnosis associated with fibrosis and thickening of the gall bladder in the setting of cholelithiasis and mechanical irritation in the setting of recurrent attacks of acute cholecystitis.
- Clinically “chronic” cholecystitis can refer to recurrent attacks of RUQ pain in a patient with a history of cholecystitis.
- Acalculous cholecystitis occurs in about 10–15% of patients presenting with acute cholecystitis. These patients are often hospitalized and severely debilitated.
Incidence/prevalence
- Acute calculous cholecystitis affects about 20% of patients with untreated symptomatic biliary colic. Most patients are women but disease presentation tends to be more severe in men. Patients with cholelithiasis and underlying diabetes mellitus are more likely to develop cholecystitis and more likely to have complicated disease.
- Cholangitis occurs in <1% of symptomatic patients with radiologic evidence of choledocholithiasis.
- Prior to the advent of HAART, the incidence of AIDS cholangiopathy approached 26% in patients with AIDS and CD4 counts of <100/mm3 during outbreaks of cryptosporidiosis.
Etiology
- The vast majority of cases of acute cholecystitis and cholangitis are associated with cholelithisasis and with obstruction of the cystic duct or the common bile duct respectively.
- Most reported cases of AIDS cholangiopathy are associated with intestinal infections with Cryptosporidium parvum, Microsporidium (e.g. Encephalitozoon intestinalis), or Cyclospora cayetanensis. CMV has also been associated with AIDS cholangiopathy.
Pathology/pathogenesis
- In patients with cholelithiasis, transient obstruction of the cystic duct may result in pain or biliary colic. Prolonged impaction, however, can result in distension and inflammation of the gall bladder or cholecystitis. This often begins as a sterile process but can progress to infection with enteric flora including Enterobacteriaceae (e.g. Escherichia coli and Klebsiella pneumoniae) as well as enterococci and anaerobes.
- With incompetence of the ampulla (e.g. after stone passage, sphincterotomy or cannulation of the biliary tree, i.e. after an ERCP) bacteria can enter the biliary system from the gut (ascending cholangitis). Impedance of the mechanical flow of bile can lead to acute cholangitis. Enteric organisms are the most common pathogens implicated in ascending cholangitis.
- The pathogenesis of AIDS cholangiopathy remains unclear. C. parvum appears to have a tropism for bile ducts. Histopathologic specimens often reveal non-specific inflammation and changes similar to those described with PSC are noted surrounding the portal tracts.
Section 2: Prevention
Clinical Pearls
- In the setting of symptomatic cholelithiasis, cholecystectomy is commonly employed to prevent future episodes of cholecystitis and/or cholangitis.
- In patients with a history of HIV infection, compliance with antiretroviral therapy is essential in preventing opportunistic infections and AIDS cholangiopathy as this syndrome is rare in patients without low CD4 counts.
Screening
- Routine screening for cholelithiasis, choledocholithiasis, and AIDS cholangiopathy in asymptomatic patients is not recommended.
Primary prevention
- Prevention of both cholecystitis and cholangitis can be achieved by treating biliary obstruction. Cholecystectomy is recommended for prevention of cholecystitis in patients with symptomatic cholelithiasis.
- Initiation of and compliance with HAART may prevent AIDS cholangiopathy, since the disease entity is rare in patients with elevated CD4 counts.
Secondary prevention
- In patients with a history of cholangitis secondary to mechanical obstruction by gallstones, a cholecystectomy is recommended to prevent recurrent episodes.
- The incidence of ascending cholangitis post-ERCP can be decreased by appropriate disinfection, peri-procedural antimicrobial prophylaxis in patients with incomplete drainage, stent placement in patients with incomplete drainage, and timely stent exchange.
- In patients with choledocholithiasis and cholangitis who are not candidates for cholecystectomy, endoscopic sphincterotomy provides partial protection against recurrent disease.
Section 3: Diagnosis
Clinical Pearls
- A detailed history regarding fevers and pain including onset, frequency and quality, as well as any recent procedures, aid in determining the etiology of a biliary infection.
- Charcot’s triad (jaundice, RUQ pain and fever), initially described in 1877, remains the clinical standard for diagnosing ascending cholangitis. Confirmation of this diagnosis includes aspiration of purulent biliary fluid during an ERCP or percutaneous or surgical biliary decompression. ERCP in the case of cholangitis serves as both a diagnostic and therapeutic tool.
- Ultrasound and hepatobiliary scintigraphy (e.g. HIDA scan) are the most common imaging studies employed to diagnose acute cholecystitis.
- AIDS cholangiopathy should be considered in the appropriate patient (HIV infection with CD4 count <100/mm3) with a history of right upper quadrant pain and diarrhea. ERCP is often diagnostic.
Typical presentation
- Acute cholecystitis usually presents with severe pain localizing to the RUQ. Fevers are frequent, but frank jaundice is rare. Jaundice, however, is a hallmark of cholangitis along with RUQ pain and fevers.
- AIDS cholangiopathy often presents in patients with low CD4 counts with non-specific abdominal discomfort, weight loss and diarrhea. Fevers and jaundice are much less common than with cholecystitis or cholangitis.
Clinical diagnosis
History
- Many patients presenting with acute cholecystitis report a history of symptoms consistent with biliary colic. This includes episodic pain localizing to the epigastrium or the RUQ. The pain frequently is exacerbated with food intake or at night. Commonly the pain will radiate to the back and be associated with nausea and vomiting. In a patient with acute cholecystitis the pain persists, is severe, and localizes to the RUQ. A history of biliary colic may also be present in patients presenting with cholangitis. Charcot’s triad – the triumvirate of pain, fever and jaundice – still remains helpful clinically in diagnosing cholangitis. A history of a recent biliary intervention (e.g. sphincterotomy or ERCP) may also contribute to the diagnosis.
- AIDS cholangiopathy should be considered in patients with low CD4 counts complaining of non-specific GI symptoms.
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