Eosinophilic Cholangitis—A Challenging Diagnosis of Benign Biliary Stricture

Abstract

When confronting a biliary stricture, both benign and malignant etiologies must be carefully considered as a variety of benign biliary strictures can masquerade as hilar cholangiocarcinoma (CCA). Therefore, patients could undergo a major surgery despite the possibility of a benign biliary disease. Approximately 15% to 24% of patients undergoing surgical resection for suspected biliary malignancy will have benign pathology. Eosinophilic cholangitis (EC) is a rare benign disorder of the biliary tract, which can cause obstructive jaundice and can pose a difficult diagnostic task.We present a rare case of a young woman who was referred to our hospital with obstructive painless jaundice due to a biliary stricture at the confluence of the hepatic bile ducts, with a provisional diagnosis of cholangiocarcinoma.Though, during her work up she was found to have EC, an extremely rare benign cause of biliary stricture, which is characterized by a dense eosinophilic infiltration of the biliary tree causing stricturing, fibrosis, and obstruction and which is reversible with short-term high-dose steroids.Despite its rarity, EC should be taken into consideration when imaging modalities demonstrate a biliary stricture, especially if preoperative diagnosis of malignancy cannot be made, in the setting of peripheral eosinophilia and the absence of cardinal symptoms of malignancy.

The Great Imposture: Eosinophilic Cholangitis

“…Eosinophilic Cholangitis (EC) is a rare, benign disease of transmural eosinophilic infiltration of the biliary tree leading to stricture formation and obstruction [1,3]. Most cases present as a focal process within the biliary system, although there have been a few reported cases of concomitant disease in the kidneys, ureters and pericardium [4].…”

“…in 1985 who reported a case of a thickened gallbladder wall and stenosis of intrahepatic bile ducts with eosinophilic infiltration in the cystic duct, gallbladder, lymph nodes and bone marrow, consistent with EC [4,5]. EC is hypothesized to originate from increased IgE, IL-5 levels leading to diffuse eosinophilic infiltration of the biliary system with TGF-ß produced by eosinophils leading to fibrosis and subsequent stricture formation of the biliary tract [1,6,7]. While the inciting cause is unknown, EC has also been proposed as a possible extension of cholecystitis secondary to infections caused by Enterobacter aerogenes or Candida albicans [8].…”

“…Eosinophilic Cholangitis (EC) is described as an eosinophilic infiltrative process of the biliary tree resulting in fibrosis, stricture, obstruction and often presenting with a tumor-like appearance. EC is primarily identified as an isolated lesion and may also present with concomitant peripheral eosinophilia and/or extra-biliary involvement of the kidneys, ureters, pancreas or lymph nodes [1]. These rare lesions are often mistaken clinically and radiologically for cholangiocarcinoma, resulting in patients undergoing extensive surgical resections for presumed malignancy [2].…”

“…These rare lesions are often mistaken clinically and radiologically for cholangiocarcinoma, resulting in patients undergoing extensive surgical resections for presumed malignancy [2]. If a diagnosis can be confirmed, however, patients may be successfully medically managed [1].…”

Immunosuppression as effective therapy for eosinophilic cholangiopathy: A case series and review of the literature

“…EC has been first described in 1985 by Butler et al, since then, a total of 35 patients have been reported (details in Table ). We provide additional five cases of EC, which is the largest single patient series reported.…”