Eosinophilic cholangitis is a potentially underdiagnosed etiology in indeterminate biliary stricture

Abstract

AIMTo investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS).METHODSAll 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.RESULTSone 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).CONCLUSIONEC is an underdiagnosed benign etiology of SC and IBS, which has to be considered in differential diagnosis of IBS.

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

“…In a recent retrospective study by Walter et al, histopathological samples (eg, biopsies and surgical specimens) of patients with a diagnosis of sclerosing cholangitis were investigated. In three of 135 patients, a post‐hoc diagnosis of EC was made, suggesting that EC is an underdiagnosed benign aetiology of sclerosing cholangitis and indeterminate biliary stricture …”

Eosinophilic cholangitis treatment with budesonide

“…EC is a benign condition and should be managed with a trial of corticosteroids before considering more invasive treatment. A recent retrospective study showed an EC prevalence of 2.2% from a cohort of 135 cases of sclerosing cholangitis and post-hoc diagnosis of EC was ascertained in 30% (3/10) of patients where no cause of indeterminate biliary stricture was identified[ 8 ]. Our patient was on oral budesonide treatment for 9 mo with biochemical resolution of her eosinophilia and liver function test.…”

Endoskopische Diagnostik der unklaren Gallengangsstenose: Aktueller Stand und Ausblick

“…Zur Diagnose einer mit IgG4 assoziierten Cholangitis sollte der Cut-off auf das Vierfache der oberen Normgrenze gelegt werden, da leicht erhöhte Werte auch bei CCA-Patienten beschrieben sind [7]. Eine periphere Eosinophilie kann auf eine eosinophile Cholangitis hinweisen [8].…”