Retrospective Study
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 14, 2017; 23(6): 1044-1050
Published online Feb 14, 2017. doi: 10.3748/wjg.v23.i6.1044
Eosinophilic cholangitis is a potentially underdiagnosed etiology in indeterminate biliary stricture
Dirk Walter, Sylvia Hartmann, Eva Herrmann, Jan Peveling-Oberhag, Wolf O Bechstein, Stefan Zeuzem, Martin-Leo Hansmann, Mireen Friedrich-Rust, Jörg G Albert
Dirk Walter, Stefan Zeuzem, Mireen Friedrich-Rust Department of Internal Medicine I, Johann Wolfgang Goethe-University Hospital, 60590 Frankfurt, Germany
Sylvia Hartmann, Martin-Leo Hansmann, Dr. Senckenberg Institute for Pathology, Johann Wolfgang Goethe-University Hospital, 60590 Frankfurt, Germany
Eva Herrmann, Institute of Biostatistics and Mathematical Modeling, Johann Wolfgang Goethe-University Hospital, 60590 Frankfurt, Germany
Jan Peveling-Oberhag, Jörg G Albert, Department for Gastroenterology, Hepatology and Endocrinology, Robert-Bosch Hospital, 70376 Stuttgart, Germany
Wolf O Bechstein, Department of General and Visceral Surgery, Johann Wolfgang Goethe-University Hospital, 60590 Frankfurt, Germany
Author contributions: Walter D and Albert JG designed the concept of the study; Data were gathered by Walter D, Peveling-Oberhag J, Bechstein WO, Zeuzem S and Friedrich-Rust M; Hartmann S and Hansmann ML performed histopathological review; Statistical analysis was performed from Herrmann E; The manuscript was drafted by Walter D, Hartmann S, Peveling-Oberhag J and Albert JG; all authors critically revised the manuscript.
Institutional review board statement: The study protocol was approved by the institutional review board (No. 478/15) of the local ethics committee of the University Hospital Frankfurt.
Informed consent statement: Patient informed consent was waived due to the retrospective design of the study.
Conflict-of-interest statement: The authors have no conflicts of interest to declare or other disclosures of record.
Data sharing statement: No additional data are available.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Dr. Dirk Walter, Department of Internal Medicine I, Johann Wolfgang Goethe-University Hospital, Theodor-Stern-Kai 7, 60590 Frankfurt, Germany.
Telephone: +49-69-63015122 Fax: +49-69-63016448
Received: October 6, 2016
Peer-review started: October 10, 2016
First decision: October 20, 2016
Revised: November 17, 2016
Accepted: January 11, 2017
Article in press: January 11, 2017
Published online: February 14, 2017


To investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS).


All patients with diagnosis of sclerosing cholangitis (SC) and histopathological samples such as biopsies or surgical specimens at University Hospital Frankfurt from 2005-2015 were included. Histopathological diagnoses as well as further clinical course were reviewed. Tissue samples of patients without definite diagnosis after complete diagnostic work-up were reviewed regarding presence of eosinophilic infiltration and IgG4 positive plasma cells. Eosinophilic infiltration was as well assessed in a control group of liver transplant donors and patients with primary sclerosing cholangitis.


one hundred and thirty-five patients with SC were included. In 10/135 (13.5%) patients, no potential cause of IBS could be identified after complete diagnostic work-up and further clinical course. After histopathological review, a post-hoc diagnosis of EC was established in three patients resulting in a prevalence of 2.2% (3/135) of all patients with SC as well as 30% (3/10) of patients, where no cause of IBS was identified. 2/3 patients with post-hoc diagnosis of EC underwent surgical resection with suspicion for malignancy. Diagnosis of IgG4-related cholangitis was observed in 7/135 patients (5.1%), whereas 3 cases were discovered in post-hoc analysis. 6/7 cases with IgG4-related cholangitis (85.7%) presented with eosinophilic infiltration in addition to IgG4 positive plasma cells. There was no patient with eosinophilic infiltration in the control group of liver transplant donors (n = 27) and patients with primary sclerosing cholangitis (n = 14).


EC is an underdiagnosed benign etiology of SC and IBS, which has to be considered in differential diagnosis of IBS.

Keywords: Indeterminate biliary stricture, endoscopy, endoscopic retrograde cholangiopancreatography, eosinophilic cholangitis, bile duct stenosis, IgG4-related disease, primary sclerosing cholangitis

Core tip: To differentiate benign from malignant disease in indeterminate biliary strictures (IBS) is crucial for clinical management. To date, data on eosinophilic cholangitis (EC) as a potential cause of IBS are lacking. In this retrospective study, we demonstrate that EC occurs in up to 30% of patients presenting with IBS and unclear clinical and histopathological findings at the end of diagnostic work-up. We thereby demonstrate that EC is a potentially underdiagnosed benign disease, which has to be considered in differential diagnoses of IBS to prevent these patients from surgery.