TY - JOUR
T1 - Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
AU - Cottin, Vincent
AU - Bel, Elisabeth
AU - Bottero, Paolo
AU - Dalhoff, Klaus
AU - Humbert, Marc
AU - Lazor, Romain
AU - Sinico, Renato A.
AU - Sivasothy, Pasupathy
AU - Wechsler, Michael E.
AU - Groh, Matthieu
AU - Marchand-Adam, Sylvain
AU - Khouatra, Chahéra
AU - Wallaert, Benoit
AU - Taillé, Camille
AU - Delaval, Philippe
AU - Cadranel, Jacques
AU - Bonniaud, Philippe
AU - Prévot, Grégoire
AU - Hirschi, Sandrine
AU - Gondouin, Anne
AU - Dunogué, Bertrand
AU - Chatté, Gérard
AU - Briault, Amandine
AU - Jayne, David
AU - Guillevin, Loïc
AU - Cordier, Jean François
N1 - Publisher Copyright:
Copyright © 2016 ERS.
Copyright:
Copyright 2018 Elsevier B.V., All rights reserved.
PY - 2016/11/1
Y1 - 2016/11/1
N2 - 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.
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.
UR - http://www.scopus.com/inward/record.url?scp=84994364950&partnerID=8YFLogxK
U2 - 10.1183/13993003.00097-2016
DO - 10.1183/13993003.00097-2016
M3 - Journal articles
C2 - 27587545
AN - SCOPUS:84994364950
SN - 0903-1936
VL - 48
SP - 1429
EP - 1441
JO - European Respiratory Journal
JF - European Respiratory Journal
IS - 5
ER -
