Titre :

Localised Versus Systemic Granulomatosis with Polyangiitis: Data from the French Vasculitis Study Group Registry.

Auteur(s) :

Iudici, Michele [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Université de Genève = University of Geneva [UNIGE]
Hôpital Cochin [AP-HP]
Pagnoux, Christian [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Hôpital Cochin [AP-HP]
Courvoisier, D. S. [Auteur]
Cohen, P. [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Hôpital Cochin [AP-HP]
Néel, A. [Auteur]
Service de médecine interne [Nantes]
Hôtel-Dieu de Nantes
Aouba, A. [Auteur]
Service de médecine interne [CHU Caen]
Lifermann, F. [Auteur]
Centre Hospitalier de Dax
Ruivard, M. [Auteur]
Service Médecine Interne - site Gabriel-Montpied [CHU Clermont-Ferrand]
Aumaître, O. [Auteur]
Bonnotte, B. [Auteur]
Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand [CHU Dijon]
Maurier, F. [Auteur]
Centre hospitalier régional Metz-Thionville [CHR Metz-Thionville]
Le Gallou, T. [Auteur]
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Karras, A. [Auteur]
Hôpital Européen Georges Pompidou [APHP] [HEGP]
Khouatra, C. [Auteur]
Hôpital Louis Pradel [CHU - HCL]
Université Claude Bernard Lyon 1 [UCBL]
Infections Virales et Pathologie Comparée - UMR 754 [IVPC]
Jourde-Chiche, N. [Auteur]
Hôpital de la Conception [CHU - APHM] [LA CONCEPTION]
Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research [C2VN]
Viallard, J. F. [Auteur]
Service de médecine interne et maladies infectieuses [Bordeaux]
Blanchard-Delaunay, C. [Auteur]
Centre Hospitalier Georges Renon [Niort] [CH Georges Renon Niort]
Godmer, Pascal [Auteur]
Centre hospitalier Bretagne Atlantique (Morbihan) [CHBA]
Université de Bretagne Sud - Vannes [UBS Vannes]
Le Quellec, A. [Auteur]
Centre Hospitalier Régional Universitaire [Montpellier] [CHRU Montpellier]
Quéméneur, T. [Auteur]
Centre hospitalier [Valenciennes, Nord]
De Moreuil, C. [Auteur]
Département de Médecine Interne et Pneumologie [Brest] [DMIP - Brest]
Régent, A. [Auteur]
Hôpital Cochin [AP-HP]
Terrier, B. [Auteur]
Hôpital Cochin [AP-HP]
Mouthon, L. [Auteur]
Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
Hôpital Cochin [AP-HP]
Guillevin, L. [Auteur]
Hôpital Cochin [AP-HP]
Puéchal, X. [Auteur]
Hôpital Cochin [AP-HP]

Titre de la revue :

Rheumatology

Nom court de la revue :

Rheumatology (Oxford)

Date de publication :

2021-09-21

ISSN :

1462-0332

Mot(s)-clé(s) en anglais :

granulomatosis with polyangiitis
ANCA-associated vasculitis
vasculitis

Discipline(s) HAL :

Sciences du Vivant [q-bio]

Résumé en anglais : [en]

Objective To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA). Methods EULAR definitions ...
Lire la suite >Objective To describe the main features at diagnosis and evolution over time of patients with localized granulomatosis with polyangiitis (L-GPA) compared with those of systemic GPA (S-GPA). Methods EULAR definitions of L-GPA, i.e. upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients’ characteristics at diagnosis and long-term outcomes were analysed and compared. Results Among the 795 Registry patients, 79 (10%) had L-GPA. Their main clinical manifestations were rhinitis, lung nodules, sinusitis and otitis. L-GPA vs S-GPA patients at diagnosis, respectively, were younger, more frequently had saddle nose deformity or subglottic stenosis and were less often PR3-ANCA–positive. L-GPA vs S-GPA induction therapy less frequently included CYC but more often a combination of MTX and glucocorticoids; 64% of MTX-treated patients experienced disease progression within 18 months post-diagnosis. L- and S-GPA patients’ estimated relapse-free–survival probabilities, relapse rates and refractory disease rates at each time point were comparable, but L-GPA patients had more frequent ENT and lung relapses, and higher overall survival rates (P<0.02). Over a median follow-up of 3.5 years, 18 (22.8%) L-GPA progressed to S-GPA, either as a relapse after a period in remission or more frequently in the context of refractory disease. L-GPA patients experienced more ENT-related damage. Conclusions The relapse risks of L-GPA and S-GPA were similar, but relapse patterns differed and L-GPA overall survival rate was higher. About one-quarter of L-GPA patients developed S-GPA over time, but without end-stage organ involvement.Lire moins >

Langue :

Anglais

Comité de lecture :

Oui

Audience :

Internationale

Vulgarisation :

Non

Établissement(s) :

Université de Lille
Inserm
CHU Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Date de dépôt :

2024-01-12T06:01:30Z
2024-03-01T14:20:54Z