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

Vincent Cottin*, Elisabeth Bel, Paolo Bottero, Klaus Dalhoff, Marc Humbert, Romain Lazor, Renato A. Sinico, Pasupathy Sivasothy, Michael E. Wechsler, Matthieu Groh, Sylvain Marchand-Adam, Chahéra Khouatra, Benoit Wallaert, Camille Taillé, Philippe Delaval, Jacques Cadranel, Philippe Bonniaud, Grégoire Prévot, Sandrine Hirschi, Anne GondouinBertrand Dunogué, Gérard Chatté, Amandine Briault, David Jayne, Loïc Guillevin, Jean François Cordier

*Corresponding author for this work
34 Citations (Scopus)

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.

Original languageEnglish
JournalEuropean Respiratory Journal
Volume48
Issue number5
Pages (from-to)1429-1441
Number of pages13
ISSN0903-1936
DOIs
Publication statusPublished - 01.11.2016

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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