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

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

Author information

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.

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

PMID:
25971154
DOI:
10.1016/j.ejim.2015.04.022
[Indexed for MEDLINE]
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