Published online Feb 14, 2017. doi: 10.3748/wjg.v23.i6.1044
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.
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.