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

Background: Eosinophilic cholangitis (EC) is a rare inflammatory disorder leading to
biliary 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 performed
using PubMed. Search keywords that were used included “eosinophilic cholangitis, ”
“eosinophilic cholangiopathy,”“eosinophilic infiltration” and “biliary obstruction.” Five
additional cases of EC treated at University Medical Center Hamburg‐Eppendorf
were analysed.
Results: Forty cases of EC were included in the analysis. Most frequent clinical
symptoms were abdominal pain (70%) and jaundice (48%). Laboratory evaluation
revealed 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ted without immunosuppression or in whom immunosuppression was withdrawn,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 patients
suffering from EC. In case of advanced hepatic damage or disease recurrence, long‐
term immunosuppression should be considered. Histology and interdisciplinary
collaboration is essential for diagnosis and management of EC