PT  - JOURNAL ARTICLE
AU  - Vincent Cottin
AU  - Elisabeth Bel
AU  - Paolo Bottero
AU  - Klaus Dalhoff
AU  - Marc Humbert
AU  - Romain Lazor
AU  - Renato A. Sinico
AU  - Pasupathy Sivasothy
AU  - Michael E. Wechsler
AU  - Matthieu Groh
AU  - Sylvain Marchand-Adam
AU  - Chahéra Khouatra
AU  - Benoit Wallaert
AU  - Camille Taillé
AU  - Philippe Delaval
AU  - Jacques Cadranel
AU  - Philippe Bonniaud
AU  - Grégoire Prévot
AU  - Sandrine Hirschi
AU  - Anne Gondouin
AU  - Bertrand Dunogué
AU  - Gérard Chatté
AU  - Amandine Briault
AU  - David Jayne
AU  - Loïc Guillevin
AU  - Jean-François Cordier
TI  - Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss)
AID  - 10.1183/13993003.00097-2016
DP  - 2016 Nov 01
TA  - European Respiratory Journal
PG  - 1429--1441
VI  - 48
IP  - 5
4099  - http://erj.ersjournals.com/content/48/5/1429.short
4100  - http://erj.ersjournals.com/content/48/5/1429.full
SO  - Eur Respir J2016 Nov 01; 48
AB  - The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail.In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease.The study population included 157 patients (mean±sd age 49.4±14.1 years), with a mean±sd blood eosinophil count of 7.4±6.4×109 L−1 at diagnosis. There was a mean±sd of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3–6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively.In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity.In EGPA, asthma is severe and progresses to persistent airflow obstruction despite corticosteroids in many patients http://ow.ly/8uf8301KSoT