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

Details

Serval ID
serval:BIB_745D2B8C6B51
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
Journal
The European respiratory journal
Author(s)
Cottin V., Bel E., Bottero P., Dalhoff K., Humbert M., Lazor R., Sinico R.A., Sivasothy P., Wechsler M.E., 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.F.
Working group(s)
   the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires (GERM“O”P)
ISSN
1399-3003 (Electronic)
ISSN-L
0903-1936
Publication state
Published
Issued date
11/2016
Peer-reviewed
Oui
Volume
48
Number
5
Pages
1429-1441
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
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±6.4×10 javax.xml.bind.JAXBElement@6693fb03  L javax.xml.bind.JAXBElement@68dfd280 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.

Keywords
Administration, Oral, Adolescent, Adrenal Cortex Hormones/administration & dosage, Adult, Aged, Aged, 80 and over, Asthma/physiopathology, Bronchoalveolar Lavage, Churg-Strauss Syndrome/complications, Churg-Strauss Syndrome/physiopathology, Eosinophilia/physiopathology, Eosinophils/cytology, Female, France, Granulomatosis with Polyangiitis/complications, Granulomatosis with Polyangiitis/physiopathology, Humans, Male, Middle Aged, Prognosis, Respiratory Function Tests, Retrospective Studies, Severity of Illness Index, Systemic Vasculitis/physiopathology, Tomography, X-Ray Computed, Treatment Outcome, Young Adult
Pubmed
Web of science
Open Access
Yes
Create date
16/09/2016 20:37
Last modification date
20/08/2019 14:32
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