Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

•Consensus guidelines for EGPA diagnosis and management are still lacking.•Twenty-three disease experts used a modified Delphi process.•Twenty-two diagnostic, evaluation, and treatment recommendations were retained.•Thirteen high-priority research topics were also identified.•Regular updates will be necessary to maintain recommendation accuracy.

ObjectiveTo develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA).MethodsThe EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached.ResultsTwenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed.DiscussionThese recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.