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.

Cottin, V., Bel, E., Bottero, P., Dalhoff, K., Humbert, M., Lazor, R., et al. (2016). Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). EUROPEAN RESPIRATORY JOURNAL, 48(5), 1429-1441 [10.1183/13993003.00097-2016].

Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

SINICO, RENATO ALBERTO;
2016

Abstract

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.
Articolo in rivista - Articolo scientifico
Pulmonary and Respiratory Medicine
English
1429
1441
13
Cottin, V., Bel, E., Bottero, P., Dalhoff, K., Humbert, M., Lazor, R., et al. (2016). Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). EUROPEAN RESPIRATORY JOURNAL, 48(5), 1429-1441 [10.1183/13993003.00097-2016].
Cottin, V; Bel, E; Bottero, P; Dalhoff, K; Humbert, M; Lazor, R; Sinico, R; Sivasothy, P; Wechsler, M; Groh, M; Marchand Adam, S; Khouatra, C; Wallaert, B; Taillé, C; Delaval, P; Cadranel, J; Bonniaud, P; Prévot, G; Hirschi, S; Gondouin, A; Dunogué, B; Chatté, G; Briault, A; Jayne, D; Guillevin, L; Cordier, J
File in questo prodotto:
File Dimensione Formato  
10281-139114.pdf

accesso aperto

Tipologia di allegato: Publisher’s Version (Version of Record, VoR)
Dimensione 436.04 kB
Formato Adobe PDF
436.04 kB Adobe PDF Visualizza/Apri
Respiratory manifestations....pdf

Solo gestori archivio

Tipologia di allegato: Publisher’s Version (Version of Record, VoR)
Dimensione 436.04 kB
Formato Adobe PDF
436.04 kB Adobe PDF   Visualizza/Apri   Richiedi una copia

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/139114
Citazioni
  • Scopus 64
  • ???jsp.display-item.citation.isi??? 64
Social impact