Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
OBJECTIVE
To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA.


METHODS
Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease.


RESULTS
The study population included 157 patients (mean age 49.4±14.1), with a follow-up of 7.4±6.4years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p=0.005) and with the presence of ANCA (p<0.001). Overall, 59% of patients had polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with polyangiitis had more systemic manifestations including arthralgias (p=0.02) and renal disease (p=0.024), had higher peripheral eosinophilia (p=0.027), and a trend towards less myocarditis (p=0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features.


CONCLUSIONS
We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations.
Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre national de référence des maladies pulmonaire rares, Université de Lyon, Université Claude Bernard Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France. Electronic address: vincent.cottin@chu-lyon.fr.
Vincent
V
Cottin
Department of Respiratory Medicine, Academic Medical Center, PO Box 22660, 1100 DD Amsterdam, The Netherlands.
Elisabeth
E
Bel
Allergy and Clinical Immunology Outpatient Clinic, Ospedale "G. Fornaroli" di Magenta, Azienda Ospedaliera di Legnano, Milan, Italy.
Paolo
P
Bottero
Medizinische Klinik III, Universitätsklinikum Schleswig Holstein, Campus Lübeck, Member of the German Center for Lung Research (DZL), D-23538, Lübeck, Germany.
Klaus
K
Dalhoff
CHU Bicêtre, service de pneumologie, 94270, Le Kremlin-Bicêtre, France.
Marc
M
Humbert
Interstitial and rare lung disease Unit, Respiratory Medicine Department, Lausanne, University Hospital, Lausanne, Switzerland.
Romain
R
Lazor
Department of Medicine and Surgery Università degli Studi di Milano - Bicocca (School of Medicine and Surgery), via Cadore, 48 - 20900 Monza (MB), Italy.
Renato A
RA
Sinico
Department of Respiratory Medicine, Addenbrookes Hospital, Cambridge University Hospitals Foundation Trust, Hills Rd, Cambridge, United Kingdom CB3 9LG.
Pasupathy
P
Sivasothy
National Jewish Health, Denver, CO, USA, and University of Colorado, Aurora, CO.
Michael E
ME
Wechsler
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université Paris Descartes, Paris, France.
Matthieu
M
Groh
CHRU Tours, service de pneumologie et université Francois Rabelais, Tours, France.
Sylvain
S
Marchand-Adam
Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre national de référence des maladies pulmonaire rares, Université de Lyon, Université Claude Bernard Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France.
Chahéra
C
Khouatra
CHRU Lille, service de pneumologie, Lille, France.
Benoit
B
Wallaert
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, 75018, Paris, France.
Camille
C
Taillé
CHU Rennes, service de pneumologie, Rennes, France.
Philippe
P
Delaval
Chest Department and Expert Center for Rare Pulmonary Diseases. APHP Hôpital Tenon and P&M Curie University Paris 6, Paris, France.
Jacques
J
Cadranel
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; Dijon, 21079, France.
Philippe
P
Bonniaud
CHU de Toulouse, service de pneumologie, Toulouse, France.
Grégoire
G
Prévot
Hôpitaux universitaires de Strasbourg, nouvel hôpital civil, service de pneumologie, centre de compétences des maladies pulmonaires rares, 1 place de l'hôpital, F-67000, Strasbourg, France.
Sandrine
S
Hirschi
CHU de Besançon, service de pneumologie, Besançon, France.
Anne
A
Gondouin
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université Paris Descartes, Paris, France.
Bertrand
B
Dunogué
Centre de Pneumologie et Allergologie Respiratoire, Caluire et Cuire, France.
Gérard
G
Chatté
CHU de Grenoble, service de pneumologie, Grenoble, France.
Christophe
C
Briault
Toronto, Canada.
Christian
C
Pagnoux
Department of Medicine, University of Cambridge, UK.
David
D
Jayne
Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université Paris Descartes, Paris, France.
Loïc
L
Guillevin
Hospices civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - centre national de référence des maladies pulmonaire rares, Université de Lyon, Université Claude Bernard Lyon I, UCBL-INRA-ENVL-EPHE, UMR754, IFR128, Lyon, France.
Jean-François
JF
Cordier
D000328:Adult; D001249:Asthma / Q000276:immunology; D015267:Churg-Strauss Syndrome / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome
D007166:Immunosuppressive Agents
ANCA; Churg-Strauss syndrome; Eosinophilic granulomatosis with polyangiitis; classification; diagnostic criteria; vasculitis