Eosinophilic Cholangitis

Abstract

See related article, Chang JY et al, on page 669 in this issue of CGH. A 29-year-old Caucasian female presented with a 1-month history of epigastric discomfort, nausea, nonbilious emesis, pruritus, and a 30-pound unintentional weight loss. Her examination was notable for scleral icterus and morbid obesity. The leukocyte count was 7.0/L (reference range [RR], 4–11) with 43% neutrophils (RR, 35%–73%), 39% lymphocytes (RR, 15%–52%), 9% monocytes (RR, 4%–13%), 9% eosinophils (RR, 0%–5%), and 0% basophils (RR, 0%–2%). The serum chemistries, blood urea nitrogen (BUN), and creatinine were all normal. The aspartate aminotransferase was 153 U/L (RR, 15–40), alanine aminotransferase was 351 U/L (RR,10–44), γ-glutamyltranspeptidase was 782 U/L (RR, 0–65), and alkaline phosphatase was 282 U/L (RR, 39–117). The total bilirubin was 8.0 mg/dL (RR, 0.4–1.4) while the direct bilirubin was 5.1 mg/dL (RR, 0.1–0.3) and the indirect bilirubin was 2.9 mg/dL. The anti-mitochondrial antibody, antismooth muscle antibody, antinuclear cytoplasmic antibody, total immunoglobulin G and immunoglobulin G 4 components all were within reference ranges. The carbohydrate antigen 19-9 was 39 U/mL (RR, 0–36). A magnetic resonance cholangiopancreatography revealed a 2-cm stricture of the mid common bile duct with the epicenter (FigureA, arrowhead) located at the cystic duct insertion. An endoscopic ultrasound (EUS) demonstrated focal thickening (FigureB, single arrow) of the mid common bile duct up to 8 mm with proximal biliary dilatation. No stones were identified in the bile duct or gallbladder. Endoscopic ultrasound-guided fine needle aspirations (FigureB, arrowheads) showed reactive bile duct epithelium but no malignancy. An endoscopic stent was placed and the patient was referred for surgical evaluation. The patient underwent an excision of the extrahepatic biliary tree with portal lymphadenectomy. The pancreas appeared normal. The biliary bifurcation was reconstructed via a Roux-en-Y biliary-enteric anastomosis. The patient's postoperative course was uneventful. The gross pathology demonstrated marked thickening up to 0.9 cm of the infundibulum of the gallbladder as well as the adjacent 2.0 cm mid common bile duct stricture. Microscopically, low magnification revealed marked transmural infiltration of inflammatory cells in the gallbladder wall (FigureC) that also extended in the cystic duct and biliary tract. This heavy inflammation was composed of mature eosinophils, which demonstrated frequent gland-destructive infiltration of eosinophils (FigureC, inset) and eosinophilic abscesses. Histopathology exam did not reveal parasites or protozoa. Eosinophilic cholangitis is often, but not always, associated with a peripheral blood eosinophillia.1Rodgers M. Allen J. Koea J. McCall J. Eosinophilic cholangitis: a case of “malignant masquerade.”.HPB (Oxford). 2001; 3: 235-239Crossref PubMed Scopus (12) Google Scholar As in this case, eosinophilic cholecystitis is often associated with eosinophilic cholangitis.2Rosengart T.K. Rotterdam H. Ranson J.H. Eosinophilic cholangitis: a self-limited cause of extrahepatic biliary obstruction.Am J Gastroenterol. 1990; 85: 582-585PubMed Google Scholar The disease process is dramatically responsive to steroids,3Jeyamani R. Ramakrishna B.S. Kurian G. Eosinophilic cholangiopathy—a report of two cases.Indian J Gastroenterol. 2007; 26: 190-191PubMed Google Scholar although most cases are not identified until the biliary stricture has been resected to rule out malignancy.1Rodgers M. Allen J. Koea J. McCall J. Eosinophilic cholangitis: a case of “malignant masquerade.”.HPB (Oxford). 2001; 3: 235-239Crossref PubMed Scopus (12) Google Scholar Important in the differential diagnosis in a patient with eosinophillia are parasites or protozoa, and allergic reactions.