Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

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• 作者：Vincent Cottin^a ; ^{vincent.cottin@chu-lyon.fr} ; Elisabeth Bel^b ; Paolo Bottero^c ; Klaus Dalhoff^d ; Marc Humbert^e ; Romain Lazor^f ; Renato A. Sinico^g ; Pasupathy Sivasothy^h ; Michael E. Wechslerⁱ ; Matthieu Groh^j ; Sylvain Marchand-Adam^k ; Chahé ; ra Khouatra^a ; Benoit Wallaert^l ; Camille Taillé ; ^m ; Philippe Delavalⁿ ; Jacques Cadranel^o ; Philippe Bonniaud^p ; Gré ; goire Pré ; vot^q ; Sandrine Hirschi^r ; Anne Gondouin^s ; Bertrand Dunogué ; ^j ; Gé ; rard Chatté ; ^t ; Christophe Briault^u ; Christian Pagnoux^v ; David Jayne^w ; Loï ; c Guillevin^j ; Jean-Franç ; ois Cordier^a ; the Groupe d&rsquo ; Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM&ldquo ; O&rdquo ; P)
• 刊名：Autoimmunity Reviews
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：16
• 期：1
• 页码：1-9
• 全文大小：781 K
• 卷排序：16

文摘

To 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 ± 6.4 years. 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.