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

TY - JOUR

T1 - Immunosuppression as effective therapy for eosinophilic cholangiopathy: 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 - 2018/11/19

Y1 - 2018/11/19

N2 - Background: Eosinophilic cholangitis (EC) is a rare inflammatory disorder leading tobiliary obstruction. Diagnosis is challenging since EC can mimic malignant disease.No standardised treatment is available so far. Aim of this case series was the analy-sis of symptoms, diagnostic and therapeutic outcomes in EC patients.Methods: A systematic research of the available scientific literature was performedusing PubMed. Search keywords that were used included “eosinophilic cholangitis, ”“eosinophilic cholangiopathy,”“eosinophilic infiltration” and “biliary obstruction.” Fiveadditional cases of EC treated at University Medical Center Hamburg‐Eppendorfwere analysed.Results: Forty cases of EC were included in the analysis. Most frequent clinicalsymptoms were abdominal pain (70%) and jaundice (48%). Laboratory evaluationrevealed abnormal liver function tests (100%) and peripheral eosinophilia (82%).Treatment consisted of immunosuppression (18 patients [45%]), surgery (12 patients[30%]) or a combination of both (nine patients [23%]). In 17 patients who were trea-ted without immunosuppression or in whom immunosuppression was withdrawn,clinical condition or laboratory tests deteriorated.Conclusions: Immunosuppression is an effective therapy in the majority of patientssuffering from EC. In case of advanced hepatic damage or disease recurrence, long‐term immunosuppression should be considered. Histology and interdisciplinarycollaboration is essential for diagnosis and management of EC

AB - Background: Eosinophilic cholangitis (EC) is a rare inflammatory disorder leading tobiliary obstruction. Diagnosis is challenging since EC can mimic malignant disease.No standardised treatment is available so far. Aim of this case series was the analy-sis of symptoms, diagnostic and therapeutic outcomes in EC patients.Methods: A systematic research of the available scientific literature was performedusing PubMed. Search keywords that were used included “eosinophilic cholangitis, ”“eosinophilic cholangiopathy,”“eosinophilic infiltration” and “biliary obstruction.” Fiveadditional cases of EC treated at University Medical Center Hamburg‐Eppendorfwere analysed.Results: Forty cases of EC were included in the analysis. Most frequent clinicalsymptoms were abdominal pain (70%) and jaundice (48%). Laboratory evaluationrevealed abnormal liver function tests (100%) and peripheral eosinophilia (82%).Treatment consisted of immunosuppression (18 patients [45%]), surgery (12 patients[30%]) or a combination of both (nine patients [23%]). In 17 patients who were trea-ted without immunosuppression or in whom immunosuppression was withdrawn,clinical condition or laboratory tests deteriorated.Conclusions: Immunosuppression is an effective therapy in the majority of patientssuffering from EC. In case of advanced hepatic damage or disease recurrence, long‐term immunosuppression should be considered. Histology and interdisciplinarycollaboration is essential for diagnosis and management of EC

U2 - https://doi.org/10.1002/ygh2.209

DO - https://doi.org/10.1002/ygh2.209

M3 - SCORING: Journal article

VL - 1

SP - 33

EP - 44

JO - GastroHep

JF - GastroHep

SN - 1478-1239

IS - 1

ER -