title: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management.
abstract: OBJECTIVE
  To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA).
  
  
  METHODS
  The 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.
  
  
  RESULTS
  Twenty-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.
  
  
  CONCLUSIONS
  These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Groh
authors.affiliation: Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.affiliation: Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Baldini
authors.affiliation: Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Bel
authors.affiliation: Allergy and Clinical Immunology Outpatient Clinic, Ospedale "G. Fornaroli" di Magenta, Azienda Ospedaliera di Legnano, Milan, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Bottero
authors.affiliation: Department of Respiratory Medicine, National Referral Center for Rare Lung Diseases, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: Medical Clinic, Department of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany.
authors.forename: Klaus
authors.initials: K
authors.lastname: Dalhoff
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dunogué
authors.affiliation: Medical Clinic, Department of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Gross
authors.affiliation: Medical Clinic, Department of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Holle
authors.affiliation: Department of Respiratory and Critical Care Medicine, National Referral Center for Severe Pulmonary Hypertension, INSERM UMR-S 999, Hôpital Bicêtre, APHP, Université Paris-Sud, 94270 Le Kremlin-Bicêtre, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Jayne
authors.affiliation: Department of Pathology and Laboratory Medicine, UNC Kidney Center, University of North Carolina, Chapel Hill, NC, USA.
authors.forename: J Charles
authors.initials: JC
authors.lastname: Jennette
authors.affiliation: Interstitial and Rare Lung Disease Unit, Department of Respiratory Medicine, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
authors.forename: Romain
authors.initials: R
authors.lastname: Lazor
authors.affiliation: Department of Internal Medicine, Hôpital Saint-Louis, Université Paris 7 René Diderot, Paris, France.
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.affiliation: Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Peter A
authors.initials: PA
authors.lastname: Merkel
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Clinical Immunology Unit and Renal Unit, Department of Medicine, Azienda Ospedaliera San Carlo Borromeo, Milan, Italy.
authors.forename: Renato Alberto
authors.initials: RA
authors.lastname: Sinico
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Specks
authors.affiliation: Nephrology Unit, University Hospital of Parma, Parma, Italy.
authors.forename: Augusto
authors.initials: A
authors.lastname: Vaglio
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, CO, USA.
authors.forename: Michael E
authors.initials: ME
authors.lastname: Wechsler
authors.affiliation: Department of Respiratory Medicine, National Referral Center for Rare Lung Diseases, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France. Electronic address: loic.guillevin@cch.aphp.fr.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D026683:Advisory Committees; D015267:Churg-Strauss Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D019468:Disease Management; D005060:Europe; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000069283:Rituximab / Q000627:therapeutic use; D014481:United States
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: Anti-neutrophil cytoplasm antibody-associated vasculitis; Asthma; Churg–Strauss syndrome; Eosinophilia; Eosinophilic granulomatosis with polyangiitis; Recommendations