title: Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
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±6.4×109L-1at 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.
authors: Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre de référence des maladies orphelines pulmonaires; Université de Lyon, Université Lyon I; UCBL-INRA-ENVL-EPHE, UMR754; IFR128; Lyon, France vincent.cottin@chu-lyon.fr.
authors: Vincent
authors: 0000-0002-5591-0955
authors: V
authors: Cottin
authors: Dept of Respiratory Medicine, Academic Medical Center, Amsterdam, The Netherlands.
authors: Elisabeth
authors: E
authors: Bel
authors: Allergy and Clinical Immunology Outpatient Clinic, Ospedale ''G. Fornaroli'' di Magenta, Azienda Ospedaliera di Legnano, Milan, Italy.
authors: Paolo
authors: P
authors: Bottero
authors: Medical University of Lübeck, Dept of Internal Medicine, Lübeck, Germany.
authors: Klaus
authors: K
authors: Dalhoff
authors: CHU Bicêtre, service de pneumologie, Le Kremlin-Bicêtre, France.
authors: Marc
authors: 0000-0003-0703-2892
authors: M
authors: Humbert
authors: Interstitial and rare lung disease unit, Respiratory Medicine Dept, University Hospital, Lausanne, Switzerland.
authors: Romain
authors: R
authors: Lazor
authors: Dept of Medicine, Clinical Immunology Unit, Azienda Ospedaliera Ospedale San Carlo Borromeo, Milan, Italy.
authors: Renato A
authors: RA
authors: Sinico
authors: Dept of Respiratory Medicine, Addenbrookes Hospital, Cambridge University Hospitals Foundation Trust, Cambridge, UK.
authors: Pasupathy
authors: P
authors: Sivasothy
authors: National Jewish Health, Denver, CO, USA.|University of Colorado, Aurora, CO, USA.
authors: Michael E
authors: ME
authors: Wechsler
authors: Dept of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors: Matthieu
authors: M
authors: Groh
authors: CHRU Tours, service de pneumologie et université Francois Rabelais Tours, Tours, France.
authors: Sylvain
authors: S
authors: Marchand-Adam
authors: Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre de référence des maladies orphelines pulmonaires; Université de Lyon, Université Lyon I; UCBL-INRA-ENVL-EPHE, UMR754; IFR128; Lyon, France.
authors: Chahéra
authors: C
authors: Khouatra
authors: CHRU Lille, service de pneumologie, Lille, France.
authors: Benoit
authors: B
authors: Wallaert
authors: INSERM U1152; Univ Paris Diderot, Sorbonne Paris Cité; Assistance Publique - Hôpitaux de Paris, Hôpital Bichat, Service de Pneumologie A; Département hospitalo-universitaire FIRE, Competence Center for Rare Pulmonary Diseases, Paris, France.
authors: Camille
authors: C
authors: Taillé
authors: CHU Rennes, service de pneumologie, Rennes, France.
authors: Philippe
authors: P
authors: Delaval
authors: Chest Dept and Expert Center for Rare Pulmonary Diseases, APHP Hôpital Tenon and P&M Curie University Paris 6, Paris, France.
authors: Jacques
authors: J
authors: Cadranel
authors: Service de Pneumologie et Soins Intensifs Respiratoires, Centre Hospitalier universitaire (CHU) François Mitterrand, Inserm U866, Université de Bourgogne, Faculté de Médecine et Pharmacie, Dijon, France.
authors: Philippe
authors: P
authors: Bonniaud
authors: CHU de Toulouse, service de pneumologie, Toulouse, France.
authors: Grégoire
authors: G
authors: Prévot
authors: CHU de Strasbourg, service de pneumologie, Strasbourg, France.
authors: Sandrine
authors: S
authors: Hirschi
authors: CHU de Besançon, service de pneumologie, Besançon, France.
authors: Anne
authors: A
authors: Gondouin
authors: University of Colorado, Aurora, CO, USA.
authors: Bertrand
authors: B
authors: Dunogué
authors: Centre de Pneumologie et Allergologie Respiratoire, Caluire et Cuire, France.
authors: Gérard
authors: G
authors: Chatté
authors: CHU de Grenoble, service de pneumologie, Grenoble, France.
authors: Amandine
authors: A
authors: Briault
authors: Dept of Medicine, University of Cambridge, UK.
authors: David
authors: D
authors: Jayne
authors: AP-HP, hôpital Cochin, université Paris-Descartes, Sorbonne Paris Cité, service de médecine interne, Inserm 1016, Paris, France.
authors: Loïc
authors: L
authors: Guillevin
authors: Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre de référence des maladies orphelines pulmonaires; Université de Lyon, Université Lyon I; UCBL-INRA-ENVL-EPHE, UMR754; IFR128; Lyon, France.
authors: Jean-François
authors: JF
authors: Cordier
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001249:Asthma / Q000503:physiopathology; D018893:Bronchoalveolar Lavage; D015267:Churg-Strauss Syndrome / Q000150:complications / Q000503:physiopathology*; D004802:Eosinophilia / Q000503:physiopathology; D004804:Eosinophils / Q000166:cytology*; D005260:Female; D005602:France; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index; D056647:Systemic Vasculitis / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones