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

Matthieu Groh; Christian Pagnoux; Chiara Baldini; Elisabeth Bel; Paolo Bottero; Vincent Cottin; Klaus Dalhoff; Bertrand Dunogué; Wolfgang Gross; Julia Holle; Marc Humbert; David Jayne; J Charles Jennette; Romain Lazor; Alfred Mahr; Peter A Merkel; Luc Mouthon; Renato Alberto Sinico; Ulrich Specks; Augusto Vaglio; Michael E Wechsler; Jean-François Cordier; Loïc Guillevin

doi:10.1016/j.ejim.2015.04.022

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

Eur J Intern Med. 2015 Sep;26(7):545-53. doi: 10.1016/j.ejim.2015.04.022. Epub 2015 May 9.

Authors

Matthieu Groh¹, Christian Pagnoux², Chiara Baldini³, Elisabeth Bel⁴, Paolo Bottero⁵, Vincent Cottin⁶, Klaus Dalhoff⁷, Bertrand Dunogué¹, Wolfgang Gross⁷, Julia Holle⁷, Marc Humbert⁸, David Jayne⁹, J Charles Jennette¹⁰, Romain Lazor¹¹, Alfred Mahr¹², Peter A Merkel¹³, Luc Mouthon¹, Renato Alberto Sinico¹⁴, Ulrich Specks¹⁵, Augusto Vaglio¹⁶, Michael E Wechsler¹⁷, Jean-François Cordier⁶, Loïc Guillevin¹⁸

Affiliations

¹ 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.
² Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
³ Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy.
⁴ Department of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands.
⁵ Allergy and Clinical Immunology Outpatient Clinic, Ospedale "G. Fornaroli" di Magenta, Azienda Ospedaliera di Legnano, Milan, Italy.
⁶ Department of Respiratory Medicine, National Referral Center for Rare Lung Diseases, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon, France.
⁷ Medical Clinic, Department of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany.
⁸ 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.
⁹ Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, United Kingdom.
¹⁰ Department of Pathology and Laboratory Medicine, UNC Kidney Center, University of North Carolina, Chapel Hill, NC, USA.
¹¹ Interstitial and Rare Lung Disease Unit, Department of Respiratory Medicine, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
¹² Department of Internal Medicine, Hôpital Saint-Louis, Université Paris 7 René Diderot, Paris, France.
¹³ Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA.
¹⁴ Clinical Immunology Unit and Renal Unit, Department of Medicine, Azienda Ospedaliera San Carlo Borromeo, Milan, Italy.
¹⁵ Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA.
¹⁶ Nephrology Unit, University Hospital of Parma, Parma, Italy.
¹⁷ Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, CO, USA.
¹⁸ 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.

PMID: 25971154
DOI: 10.1016/j.ejim.2015.04.022

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.

Discussion: These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Keywords: Anti-neutrophil cytoplasm antibody-associated vasculitis; Asthma; Churg–Strauss syndrome; Eosinophilia; Eosinophilic granulomatosis with polyangiitis; Recommendations.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Advisory Committees
Churg-Strauss Syndrome / diagnosis*
Churg-Strauss Syndrome / drug therapy*
Consensus
Disease Management
Europe
Glucocorticoids / therapeutic use
Granulomatosis with Polyangiitis / diagnosis*
Granulomatosis with Polyangiitis / drug therapy*
Humans
Immunosuppressive Agents / therapeutic use
Rituximab / therapeutic use
United States

Substances

Glucocorticoids
Immunosuppressive Agents
Rituximab