Title :

Granulomatosis with polyangiitis: study of 795 patients from the french vasculitis study group registry

Author(s) :

Iudici, Michele [Auteur]
Geneva University Hospitals and Geneva University
Hôpital Cochin [AP-HP]
Pagnoux, Christian [Auteur]
Hôpital Cochin [AP-HP]
Courvoisier, Delphine S. [Auteur]
Hôpitaux universitaires de Genève = University Hospitals of Geneva [HUG]
Cohen, Pascal [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Hôpital Cochin [AP-HP]
Hamidou, Mohamed [Auteur]
Hôtel-Dieu de Nantes
Aouba, Achille [Auteur]
Hôpital Côte de Nacre [CHU Caen]
Lifermann, François [Auteur]
Centre Hospitalier de Dax
Ruivard, Marc [Auteur]
CHU Clermont-Ferrand
Aumaitre, Olivier [Auteur]
Service Médecine Interne - site Gabriel-Montpied [CHU Clermont-Ferrand]
Bonnotte, Bernard [Auteur]
Hôpital du Bocage
Maurier, Francois [Auteur]
Centre hospitalier régional Metz-Thionville [CHR Metz-Thionville]
Decaux, Olivier [Auteur]
Centre Hospitalier Universitaire [Rennes]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Karras, Alexandre [Auteur]
Service de Néphrologie et Hémodialyse [CHU HEGP]
Khouatra, Chahera [Auteur]
Hôpital Louis Pradel [CHU - HCL]
Infections Virales et Pathologie Comparée - UMR 754 [IVPC]
Jourde-Chiche, Noémie [Auteur]
Hôpital de la Conception [CHU - APHM] [LA CONCEPTION]
Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research [C2VN]
Viallard, Jean-Francois [Auteur]
Hôpital Haut-Lévêque [CHU Bordeaux]
Blanchard-Delaunay, Claire [Auteur]
Centre Hospitalier Georges Renon [Niort] [CH Georges Renon Niort]
Godmer, Pascal [Auteur]
Centre hospitalier Bretagne Atlantique (Morbihan) [CHBA]
Le Quellec, Alain [Auteur]
Hôpital Saint Eloi [CHU Montpellier]
Quemeneur, Thomas [Auteur]
Centre hospitalier [Valenciennes, Nord]
De Moreuil, Claire [Auteur]
Hôpital de la Cavale Blanche - CHRU Brest [CHU - BREST ]
Regent, Alexis [Auteur]
Hôpital Cochin [AP-HP]
Terrier, Benjamin [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Hôpital Cochin [AP-HP]
Mouthon, Luc [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Hôpital Cochin [AP-HP]
Guillevin, Loic [Auteur]
Hôpital Cochin [AP-HP]
Puechal, Xavier [Auteur]
Centre de référence des maladies auto-immunes systémiques rares d'Île-de-France / National Reference Center for Rare Systemic Autoimmune Diseases
Hôpital Cochin [AP-HP]

Journal title :

Seminars in Arthritis and Rheumatism

Abbreviated title :

Semin Arthritis Rheum

Volume number :

51

Pages :

339-346

Publication date :

2021-02-10

ISSN :

1532-866X

Keyword(s) :

Vasculitis
Granulomatosis with polyangiitis
ANCA-associated vasculitis

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database. Patients' clinical and laboratory characteristics, Birmingham ...
Show more >To describe the characteristics and long-term outcomes of patients with granulomatosis with polyangiitis (GPA) from the French Vasculitis Study Group database. Patients' clinical and laboratory characteristics, Birmingham Vasculitis Activity Score (BVAS)-assessed disease activity, malignancies, opportunistic infections, and vital status were collected at diagnosis and each visit. Estimated probabilities and predictors of overall (OS) and relapse-free survival (RFS) were analyzed by Cox regression. We enrolled 795 newly diagnosed patients, followed for a median of 3.5 years. Initial clinical manifestations involved ear, nose & throat (ENT; 80%), lungs (68%) and kidneys (56%). Among the 728 available ELISA results, 75.0% were PR3-ANCA-positive, 16.5% MPO-ANCA-positive and 62 (8.5%) ANCA-negative. Relapses occurred in 394 (50%) patients, involving ≥1 organ(s) affected at onset in 179 (46%), mainly ENT, lungs and kidneys, with mean BVAS 10.2 points below that at diagnosis (p<0.001). Five- and 10-year RFS rates were 37% and 17%, respectively. PR3-ANCA-positivity independently predicted relapse (p = 0.05) and prolonged survival (p = 0.038). OS-but not RFS-improved significantly over time (p<0.001); 10-year OS reached 88.2% (95% CI 83.9 to 92.7) for the 660 patients diagnosed after 2000. Infections were the main causes of death. Malignancy or opportunistic infection each occurred in ≤5% of the patients. Survival has improved dramatically over the last decades but the high relapse rate remains a major concern for GPA patients, once again stressing the need for therapeutic strategy optimization to lower it. PR3-ANCA-positivity was associated with increased probability of relapse and survival.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

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

Submission date :

2021-07-06T12:50:45Z
2024-03-04T13:43:57Z