Eosinophilic granulomatosis with polyangiitis (formerly Churg–Strauss syndrome): where are we now?
Authors: Matthieu Groh, Christian Pagnoux, Loïc Guillevin
Journal: European Respiratory Journal (2015)

Antineutrophil-cytoplasm antibody (ANCA)-associated vasculitides (AAV), classified as small-sized vessel vasculitides, include: granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis disease), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg–Strauss syndrome) [1]. Although they share some common features, EGPA has certain specificities, namely asthma, blood and tissue eosinophilia, and frequent ear, nose and throat (ENT) involvement, while ANCA (targeting neutrophil myeloperoxidase) are found in only a subset of patients (30–70%) [2, 3]. Moreover, EGPA diagnosis can be challenging, as its manifestations can overlap with those of primary hypereosinophilic syndromes (HES) [4]. The EGPA Consensus Task Force's first recommendations for EGPA diagnosis and individualised patient management <http://ow.ly/RZ3Sw> Members of the EGPA Task Force and co-authors of the article cited in this editorial are: Chiara Baldini (Rheumatology Unit, Dept of Internal Medicine, University of Pisa, Pisa, Italy); Elisabeth Bel (Dept of Respiratory Medicine, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands); Paolo Bottero (Allergy and Clinical Immunology Outpatient Clinic, Ospedale "G. Fornaroli" di Magenta, Azienda Ospedaliera di Legnano, Milan, Italy); Jean-François Cordier (Dept of Respiratory Medicine, National Referral Center for Rare Lung Diseases, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon, France); Vincent Cottin (Dept of Respiratory Medicine, National Referral Center for Rare Lung Diseases, Hôpital Louis-Pradel, Hospices Civils de Lyon, Lyon, France); Klaus Dalhoff (Medical Clinic, Dept of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany); Bertrand Dunogué (Dept of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France); Wolfgang Gross (Medical Clinic, Dept of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany); Julia Holle (Medical Clinic, Dept of Rheumatology, Vasculitis Center, University Clinic of Schleswig-Holstein, Lübeck and Bad Bramstedt, Germany); Marc Humbert (Dept of Respiratory and Critical Care Medicine, National Referral Center for Severe Pulmonary Hypertension, INSERM UMR-S 999, Hôpital Bicêtre, APHP, Université Paris-Sud, Le Kremlin-Bicêtre, France); David Jayne (Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK); J. Charles Jennette (Dept of Pathology and Laboratory Medicine and UNC Kidney Center, University of North Carolina, Chapel Hill, NC, USA); Romain Lazor (Interstitial and Rare Lung Disease Unit, Dept of Respiratory Medicine, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland); Alfred Mahr (Dept of Internal Medicine, Hôpital Saint-Louis, Université Paris 7 René Diderot, Paris, France); Peter A. Merkel (Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, USA); Luc Mouthon (Dept of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France); Renato Alberto Sinico (Clinical Immunology Unit and Renal Unit, Dept of Medicine, Azienda Ospedaliera San Carlo Borromeo, Milan, Italy); Ulrich Specks (Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA); Augusto Vaglio (Nephrology Unit, University Hospital of Parma, Parma, Italy); and Michael E. Wechsler (Division of Pulmonary, Critical Care and Sleep Medicine, National Jewish Health, Denver, CO, USA).