Eosinophilic cholangiopathy: the diagnostic dilemma of a recurrent biliary stricture. Should surgery be offered for all?

Abstract

SUMMARYA 63-year-old man presented with the initial diagnosis of autoimmune pancreatitis with obstructive jaundice. CT of the abdomen revealed an oedematous pancreas and dilated common bile duct (CBD), without gallstones. After failure of initial retrograde cholangiopancreatography, a percutaneous biliary catheter was inserted with good drainage. Subsequent endoscopic retrograde cholangiopancreatography (ERCP) revealed a 2 cm distal CBD stricture. A biliary stent was inserted past the stricture. Biopsy of the stricture, brush cytology of the bile duct and fine needle aspiration of pancreatic head under endoscopic ultrasound guidance were negative for malignancy. Autoimmune screen was negative as well. However, the patient represented with cholangitis requiring repeat ERCP and insertion of a second biliary stent. He finally underwent cholecystectomy with excision of the distal CBD and Roux-En-Y hepaticojejunostomy. Histology revealed diffuse eosinophilic cholecystitis and cholangitis. A retrospective review of the blood results showed persistent eosinophilia in full blood count measurements from presentation and persisting throughout the treatment period. BACKGROUND

Eosinophilic cholangitis coexisted with idiopathic thrombocytopenic purpura: Report of a case

“…Among the reported cases of eosinophilic cholangitis, 18 underwent an operation and 13 did not (Table ) . A total of 12 resected cases were diagnosed as suspected cancer of the bile tract upon the identification of local wall thickening or stricture of the bile duct, and five resected cases were diagnosed as cholecystitis with diffuse biliary stricture.…”

Eosinophilic Cholangitis Without Biliary Stricture After the Treatment of Eosinophilic Esophagitis

“…Eosinophilic cholangitis (EC) is a rare disease that can result in biliary obstruction. 1 EC exhibits similar imaging findings, such as biliary stricture and wall thickness of the bile duct, to cholangiocarcinoma, primary sclerosing cholangitis (SC), and immunoglobulin G4 (IgG4)-related SC. 2 We want to emphasize that EC is a rare entity that is overlooked, but it is important to consider in the differential because EC can be treated with steroids favorably, and unnecessary surgery can be avoided.…”

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

“…Histopathological hallmark is a dense transmural eosinophilic infiltration . “ Malignant masquerade ,” “ diagnostic dilemma ,” and “ sheep in wolves clothing ” are descriptive terms that have been used with regard to the clinical and radiological presentation of EC. Despite excessive diagnostic work‐up, EC can be indistinguishable from malignant diseases and sclerosing cholangitis .…”

“…Published treatment options ranged from symptomatic or endoscopic treatment, over immunosuppression to major surgical interventions (eg, Roux‐en‐Y‐hepaticojejunostomy). While some authors proclaim, that surgery is a definite therapy for EC, others consider EC as an inflammatory disorder with risk of recurrence and need for immunosuppressive treatment . The disease has also been suggested to be self‐limiting, but there is scarce data supporting this hypothesis.…”

“…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.…”