Immunosuppression as efficient therapy for Eosinophilic Cholangitis: A case series and review of the literature

TY - CHAP

T1 - Immunosuppression as efficient therapy for Eosinophilic Cholangitis: A case series and review of the literature

AU - Reher, Dominik

AU - Schramm, Christoph

AU - Brinkert, Florian

AU - Weidemann, Sören Alexander

AU - Plauth, Mathias

AU - Lohse, Ansgar Wilhelm

AU - Weiler-Normann, Christina

PY - 2018/1/26

Y1 - 2018/1/26

N2 - Background and Aims: Eosinophilic cholangitis (EC) is a rare benign cause for biliary obstruction. Diagnosis is challenging since EC can mimic malignant diseases and sclerosing cholangitis. Thus, in many cases EC has been diagnosed retrospectively after surgery. In other cases, diagnosis has been made after histopathological analysis of biopsies from the biliary tract or after observation of sclerosing cholangitis with concomitant marked peripheral eosinophilia. Guidelines for medical treatment are not established. Due to this fact, therapeutic interventions reported in case reports ranged from symptomatic and endoscopic treatment, over immunosuppression to major surgical interventions. Aim of this case series is the analysis of symptoms, diagnostic and therapeutic outcomes in EC patients.Methods: A systematic research of the available scientific literature was performed using PubMed. Search keywords that were used included “eosinophilic cholangitis”, “eosinophilic cholangiopathy”, “eosinophilic infiltration”, “biliary obstruction” and “treatment”. Four additional cases of EC treated at University Medical Center Hamburg-Eppendorf were analysed.Results: In total 39 cases of EC were analysed. Most frequent symptoms on clinical presentation were abdominal pain (69%) and jaundice (46%). Laboratory evaluation revealed elevation of liver function tests (82%) and peripheral eosinophilia (67%). 17 patients (44%) were treated with steroids. Duration and dosage differed significantly and in 10 patients recurrence could be observed after steroids were tapered or ceased. 12 patients (31%) were treated surgically, recurrence could be observed in one patient. 9 patients (23%) received surgical and immunosuppressive treatment, recurrence could be observed in 5 cases. In 3 of these 5 cases surgical therapy alone was insufficient and treatment with steroids was initiated consecutively. In the remaining 2 cases recurrence occurred after steroids were ceased. Taken together, in 16 patients clinical condition or laboratory tests deteriorated during clinical course. In all cases where recurrence could be observed, immunosuppression was tapered, ceased or had not been initiated in the first place.Conclusions: Immunosuppression is an efficient therapy in the majority of patients suffering from EC. In case of advanced hepatic damage or disease recurrence, a lifelong immunosuppression should be considered. Due to heterogeneous clinical presentation, radiological findings and laboratory tests, taking biopsies and interdisciplinary collaboration is essential for diagnosis and management of EC.

AB - Background and Aims: Eosinophilic cholangitis (EC) is a rare benign cause for biliary obstruction. Diagnosis is challenging since EC can mimic malignant diseases and sclerosing cholangitis. Thus, in many cases EC has been diagnosed retrospectively after surgery. In other cases, diagnosis has been made after histopathological analysis of biopsies from the biliary tract or after observation of sclerosing cholangitis with concomitant marked peripheral eosinophilia. Guidelines for medical treatment are not established. Due to this fact, therapeutic interventions reported in case reports ranged from symptomatic and endoscopic treatment, over immunosuppression to major surgical interventions. Aim of this case series is the analysis of symptoms, diagnostic and therapeutic outcomes in EC patients.Methods: A systematic research of the available scientific literature was performed using PubMed. Search keywords that were used included “eosinophilic cholangitis”, “eosinophilic cholangiopathy”, “eosinophilic infiltration”, “biliary obstruction” and “treatment”. Four additional cases of EC treated at University Medical Center Hamburg-Eppendorf were analysed.Results: In total 39 cases of EC were analysed. Most frequent symptoms on clinical presentation were abdominal pain (69%) and jaundice (46%). Laboratory evaluation revealed elevation of liver function tests (82%) and peripheral eosinophilia (67%). 17 patients (44%) were treated with steroids. Duration and dosage differed significantly and in 10 patients recurrence could be observed after steroids were tapered or ceased. 12 patients (31%) were treated surgically, recurrence could be observed in one patient. 9 patients (23%) received surgical and immunosuppressive treatment, recurrence could be observed in 5 cases. In 3 of these 5 cases surgical therapy alone was insufficient and treatment with steroids was initiated consecutively. In the remaining 2 cases recurrence occurred after steroids were ceased. Taken together, in 16 patients clinical condition or laboratory tests deteriorated during clinical course. In all cases where recurrence could be observed, immunosuppression was tapered, ceased or had not been initiated in the first place.Conclusions: Immunosuppression is an efficient therapy in the majority of patients suffering from EC. In case of advanced hepatic damage or disease recurrence, a lifelong immunosuppression should be considered. Due to heterogeneous clinical presentation, radiological findings and laboratory tests, taking biopsies and interdisciplinary collaboration is essential for diagnosis and management of EC.

U2 - DOI: 10.1055/s-0037-1612727

DO - DOI: 10.1055/s-0037-1612727

M3 - Conference contribution - Poster

VL - 56

SP - E2-E89

BT - Zeitschrift für Gastroenterologie

PB - Georg Thieme Verlag KG

T2 - German Association for the study of the Liver - Jahrestagung 2018

Y2 - 26 January 2018 through 27 January 2018

ER -