• Vincent Cottin, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A Sinico, Pasupathy Sivasothy, Michael E Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne Gondouin, Bertrand Dunogué, Gérard Chatté, Christophe Briault, Christian Pagnoux, David Jayne, Loïc Guillevin, Jean-François Cordier, and Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P).
• Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre national de référence des maladies pulmonaire rares, Université de Lyon, Université Claude Bernard Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France. Electronic address: vincent.cottin@chu-lyon.fr.
• Autoimmun Rev. 2017 Jan 1; 16 (1): 1-9.

ObjectiveTo guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA.MethodsOrgan manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease.ResultsThe study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features.ConclusionWe suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations.Copyright © 2016 Elsevier B.V. All rights reserved.

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