Gastroenterology and Hepatology Board Review: Pearls of Wisdom, Third Edition

Section VI GALLBLADDER, BILE DUCTS, AND PANCREAS

CHAPTER 34. Gallbladder and Bile Duct Miscellaneous Inflammatory Diseases

Isaac Raijman, MD

Image What biliary tract disease is associated with the acquired immunodeficiency syndrome (AIDS)?

AIDS can produce a sclerosing cholangitis-like picture associated with upper abdominal pain and elevated liver function tests, especially alkaline phosphatase. The cholangitis may be associated with the AIDS virus alone or with other infections such as cytomegalovirus (CMV), Cryptosporidium, or Microsporidia. A causative organism is found in about 60% of cases.

Image What infections may cause acalculous cholecystitis in patients with AIDS?

Most frequently CMV, but also CryptosporidiaMicrosporidia, and Isospora belli.

Image True/False: The most common infection of the bile duct in patients with AIDS is cytomegalovirus infestation.

False. The most common organism is Cryptosporidium parvum. In about 20%–40% of patients, no causative organism is found.

Image At what T lymphocyte count is AIDS cholangiopathy more likely to occur?

When the helper T-cell count is < 200.

Image What is the most likely diagnosis in a patient with AIDS who complains of severe abdominal pain and has an elevated alkaline phosphatase?

Papillary stenosis. AIDS may produce severe abdominal pain associated with elevated alkaline phosphatase due to papillary stenosis. The pain may improve dramatically after endoscopic sphincterotomy.

Image True/False: AIDS cholangiopathy adversely affects the overall outcome of AIDS patients.

False. AIDS cholangiopathy does not appear to have any influence on the progression of the underlying disease.

Image What patient group is at high risk for infectious and parasitic cholangiopathies?

Patients from Southeast Asia are particularly at risk for parasite-related bile duct disease.

Image What are the most common parasites implicated in biliary obstruction?

Clonorchis sinensis and Ascaris lumbricoides are the most frequently found parasites causing biliary disease. More frequent than stricturing is the presence of undulating and elongated filling defects of the bile ducts. Certain organisms are more specific to certain geographic areas. C. sinensis is more common in China, Japan, Vietnam, and Korea. Opistorchis felineus occurs not only in Southeast Asia but also in Siberia. Fasciola hepatica can occur anywhere in the world while F. gigantica is more common in the tropics.

Image What is Oriental cholangitis?

Oriental cholangitis is characterized by the development of pigmented stones, diffuse biliary strictures, and chronic, recurrent episodes of cholangitis. This is particularly common in people from Southeast Asia.

Image What is autoimmune cholangitis?

The clinical expression of autoimmune cholangitis is very similar to that of primary biliary cirrhosis except it is not associated with antimitochondrial antibodies.

Image What type of cholangiographic injury has been associated with intra-arterial infusion of 5-fluorodeoxyuridine?

An intrahepatic sclerosing cholangitis-type picture.

Image What conditions are associated with the development of bile duct disease causing stricture formation?

• Surgical trauma

• Anastomotic arterial strictures

• Hepatic artery thrombosis

• Biliary infections

• Biliary stenting, especially if prolonged

• Choledocholithiasis

• Bile duct ischemia

• Neoplasia

Image Jaundice occurs in what percentage of patients with acute cholecystitis without evidence of cystic duct or common bile duct obstruction?

Fifteen percent. This may be due to inflammation and swelling of the cystic duct.

Image Name five causes of secondary sclerosing cholangitis.

Operative trauma and ischemia, chronic choledocholithiasis, cholangiocarcinoma, chronic pancreatitis, and toxins such as absolute alcohol and formaldehyde.

Image Besides inflammatory bowel disease, name five chronic systemic diseases associated with sclerosing cholangitis.

Recurrent pancreatitis, diabetes mellitus, celiac disease, rheumatoid arthritis, and sarcoidosis.

Image In primary sclerosing cholangitis (PSC), how often is the pancreatic duct involved?

10%–15%.

Image Name two conditions that can mimic PSC.

Extrahepatic portal venous obstruction and metastatic cancer of the liver.

Image Describe two possible causes of obstructive jaundice in a patient with annular pancreas.

1) Recurrent pancreatitis in the head of the gland, causing edema or fibrosis that constricts the bile duct within the pancreas, and 2) fibrosis of the duodenal wall, through which the terminal portion of the bile duct passes.

Image How frequently do patients with hepatic artery aneurysms present with jaundice?

Hepatic artery aneurysms, which are situated close to the bile ducts, present with jaundice in 50% of cases.

Image Cholangitis is found in what percentage of patients with malignant strictures?

10%–15%.

Image What is the preferred treatment for lymphoma patients who present with obstructive jaundice?

Chemotherapy is the preferred treatment. Local irradiation of the hilus of the liver may be used adjunctively.

Image True/False: Bacterial involvement of the gallbladder is a primary event in the development of acute calculous cholecystitis.

False. Bacterial inflammation is considered a secondary event and is found in as many as 80% of patients with acute calculous cholecystitis undergoing cholecystectomy.

Image True/False: The presence of bacteria is uncommon in gallstone disease.

False. Approximately 70% of patients with gallstones have evidence of bacteria in the bile. Escherichia coli is the most common Gram-negative organism, whereas Enterococcus is the most common Gram-positive organism.

Image True/False: Jaundice occurs more commonly in adults with acute calculous cholecystitis compared to those with acalculous cholecystitis.

False. Approximately 20%–25% of patients with acalculous cholecystitis can have obstructive jaundice due to common bile duct obstruction from inflammatory changes. Jaundice occurs in approximately 10% of patients with calculous cholecystitis.

Image True/False: In patients with a rapidly rising bilirubin and no evidence of biliary obstruction, gallbladder perforation with secondary increased absorption of bilirubin through the peritoneal cavity should be considered.

True.

Image True/False: The most common complication of acute cholecystitis is gallbladder perforation.

False. The most common complication of acute cholecystitis is gallbladder gangrene, which can occur in about 20% of the cases. Perforation occurs in approximately 2% of the patients.

Image What is the most common location of a gallbladder perforation?

The fundus of the gallbladder due to its larger diameter and thus greater tension.

Image True/False: Hepatobiliary scanning of the gallbladder is useful in diagnosing acute acalculous cholecystitis.

True. A positive test occurs when there is no filling of the gallbladder, usually within 1 hour. However, in some normal patients, it may take up to 4 hours for the gallbladder to fill. Causes of false positives include parenteral nutrition, prolonged (> 24 hours) or limited (< 2 hours) fasting, and alcoholism.

Image What percentage of patients with acute acalculous cholecystitis develop complications?

• Gallbladder empyema (2%–12%)

• Perforation (3%–15%)

• Gangrenous cholecystitis (< 2%)

• Bleeding or hemoperitoneum (very rare)

• Emphysematous cholecystitis (usually due to Clostridium species) occurs rarely. The incidence of gangrenous gallbladder in these patients is as high as 75%.

• Septic metastases (rare)

Image What percentage of all cases of acute cholecystitis is due to acalculous disease?

Acute acalculous cholecystitis accounts for approximately 6%–17%.

Image What factors are involved in the development of acute acalculous cholecystitis?

Obstruction of the cystic duct by sludge, inspissation of bile with associated reduced flow, mechanical obstruction of the cystic duct by other diseases such as tumors or nodes, decreased gallbladder motility, systemic volume depletion, ischemia, and possibly infectious agents.

Image True/False: Infections may cause acute acalculous cholecystitis.

True. Infections are particularly important in patients with AIDS, where cytomegalovirus and Cryptosporidium play an important role. Typhoid fever is also associated with acalculous cholecystitis.

Image Acute acalculous cholecystitis may occur in those with serious injury or illness and after major complicated surgeries, particularly in the elderly. What is the cause of acute acalculous cholecystitis?

The etiology of acalculous cholecystitis is unknown but possibilities include biliary/gallbladder stasis resulting from long-standing fasting, alterations in gallbladder flow, especially in elderly patients with peripheral vascular disease, prostaglandins, and endotoxins. Gangrene, empyema, and perforation of gallbladder more commonly complicate the course of acalculous cholecystitis than acute calculous cholecystitis.

Image Acalculous cholecystitis accounts for what percentage of gallbladder perforations?

40%.

Image What diseases are associated with the development of acute acalculous cholecystitis?

Diseases associated with mesenteric vascular compromise (vasculitis), sepsis, prolonged use of total parenteral nutrition, severe burns, and intra-abdominal surgery.

Image What patient group is more commonly associated with acute acalculous cholecystitis?

Contrary to calculous disease, acalculous cholecystitis is more common in men, especially the elderly.

Image What is the overall mortality in acute acalculous cholecystitis?

Approximately 50%.

Image What is the most common cholangiographic pattern of PSC?

While it may affect both intra- and extrahepatic ducts, PSC more commonly presents as multiple short strictures found throughout the liver with characteristic beading and pruning and isolated dilations of the intrahepatic ducts.

Image True/False: Endobiliary stents are the nonsurgical treatment of choice for PSC-related bile duct strictures.

False.

Image True/False: Cholangiocarcinoma is usually easily detected in the setting of PSC.

False.

Image What is the most common cause of acute suppurative cholangitis?

Intrahepatic or extrahepatic stones are the cause of almost all cases. Patients with a biliary endoprosthesis and/or previous biliary manipulation are also at risk.

Image What is the treatment of choice in acute suppurative cholangitis?

Endoscopic decompression along with systemic antibiotics.

• • • SUGGESTED READINGS • • •

Barie PS, Eachempati SR. Acute acalculous cholecystitis. Gastroenterol Clin North Am. 2010;39(2):343-357.

Alderlieste YA, van den Elzen BD, Rauws EA, Beuers U. Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. Digestion. 2009;79(4):220-228.