Title :

Dual MPO/PR3 ANCA positivity and vasculitis: insights from a 7-cases study and an AI-powered literature review

Author(s) :

Bettacchioli, Eléonore [Auteur]
Lymphocytes B, Autoimmunité et Immunothérapies [LBAI]
Foulquier, Jean-Baptiste [Auteur]
Centre d'Investigation Clinique [Rennes] [CIC]
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Institut de recherche en santé, environnement et travail [Irset]
Chevet, Baptiste [Auteur]
CHRU Brest - Service de Rhumatologie [CHU - BREST - Rhumato]
Lymphocytes B, Autoimmunité et Immunothérapies [LBAI]
Cornec-Le Gall, Emilie [Auteur]
Génétique, génomique fonctionnelle et biotechnologies (UMR 1078) [GGB]
CHRU - Service de néphrologie, dialyse et transplantation rénale
Hanrotel, Catherine [Auteur]
Hôpital de la Cavale Blanche - CHRU Brest [CHU - BREST ]
Lanfranco, Luca [Auteur]
Université de Brest [UBO]
de Moreuil, Claire [Auteur]
Centre Hospitalier Régional Universitaire de Brest [CHRU Brest]
Groupe d'Etude de la Thrombose de Bretagne Occidentale [GETBO]
Lambert, Yannick [Auteur]
Physique - IEMN [PHYSIQUE - IEMN]
JUNIA [JUNIA]
Dueymes, Maryvonne [Auteur]
Centre Hospitalier Andrée Rosemon [Cayenne, Guyane Française]
Ecosystemes Amazoniens et Pathologie Tropicale [EPat]
Foulquier, Nathan [Auteur]
Lymphocytes B, Autoimmunité et Immunothérapies [LBAI]
Cornec, Divi [Auteur]
Université de Brest [UBO]
Lymphocytes B, Autoimmunité et Immunothérapies [LBAI]

Journal title :

Rheumatology

Pages :

2557-2568

Publisher :

Oxford University Press (OUP)

Publication date :

2024-03-29

ISSN :

1462-0324

English keyword(s) :

ANCA-associated vasculitis
MPO
PR3
double positivity

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Objectives - Anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitides (AAV) are rare conditions characterized by inflammatory cell infiltration in small blood vessels, leading to tissue necrosis. While most ...
Show more >Objectives - Anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitides (AAV) are rare conditions characterized by inflammatory cell infiltration in small blood vessels, leading to tissue necrosis. While most patients with AAV present antibodies against either myeloperoxidase (MPO) or proteinase 3 (PR3), rare cases of dual positivity for both antibodies (DP-ANCA) have been reported, and their impact on the clinical picture remains unclear. The goal of this study was to investigate the clinical implications, phenotypic profiles and outcomes of patients with DP-ANCA. Methods - A retrospective screening for DP-ANCA cases was conducted at Brest University Hospital's immunology laboratory (France), analysing ANCA results from March 2013 to March 2022. Clinical, biological, imaging, and histological data were collected for each DP-ANCA case. Additionally, a comprehensive literature review on DP-ANCA was performed, combining an artificial intelligence (AI)-based search using BIBOT software with a manual PUBMED database search. Results - The report of our cases over the last 9 years and those from the literature yielded 103 described cases of patients with DP-ANCA. We identified four distinct phenotypic profiles: (i) idiopathic AAV (∼30%); (ii) drug-induced AAV (∼25%); (iii) autoimmune disease associated with a low risk of developing vasculitis (∼20%); and (iv) immune-disrupting comorbidities (infections, cancers, etc) not associated with AAV (∼25%). Conclusion - This analysis of over a hundred DP-ANCA cases suggests substantial diversity in clinical and immunopathological presentations. Approximatively 50% of DP-ANCA patients develop AAV, either as drug-induced or idiopathic forms, while the remaining 50%, characterized by pre-existing dysimmune conditions, demonstrates a remarkably low vasculitis risk. These findings underscore the complex nature of DP-ANCA, its variable impact on patient health, and the necessity for personalized diagnostic and management approaches in these cases.Show less >

Language :

Anglais

Peer reviewed article :

Oui

Audience :

Internationale

Popular science :

Non

Collections :

• Institut d'Électronique, de Microélectronique et de Nanotechnologie (IEMN) - UMR 8520

Source :

Harvested from HAL