Can Antinuclear Antibodies Have a Pathogenic Role in Systemic Sclerosis?

Document type :

Article dans une revue scientifique: Article de synthèse/Review paper

DOI :

10.3389/fimmu.2022.930970

PMID :

Permalink :

http://hdl.handle.net/20.500.12210/100778

Title :

Can Antinuclear Antibodies Have a Pathogenic Role in Systemic Sclerosis?

Author(s) :

Chepy, Aurelien [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France [CeRAINO]
Bourel, Louisa [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Koether, Vincent [Auteur]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]
Launay, David [Auteur]

Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Dubucquoi, Sylvain [Auteur]

Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Sobanski, Vincent [Auteur]

Institut universitaire de France [IUF]
Institute for Translational Research in Inflammation - U 1286 [INFINITE]

Journal title :

Frontiers in Immunology

Abbreviated title :

Front Immunol

Volume number :

13

Pages :

930970

Publication date :

2022-07-17

ISSN :

1664-3224

English keyword(s) :

antibodies
systemic sclerosis
biomarkers
antinuclear antibodies (ANA)
pathogenic antibody

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Systemic sclerosis (SSc) is a connective tissue disease characterized by extensive fibrosis of the skin and internal organs, associated with vasculopathy and autoimmune features. Antinuclear antibodies (ANA) are found in ...
Show more >Systemic sclerosis (SSc) is a connective tissue disease characterized by extensive fibrosis of the skin and internal organs, associated with vasculopathy and autoimmune features. Antinuclear antibodies (ANA) are found in almost all SSc patients and constitute strong diagnosis and prognosis biomarkers. However, it remains unclear whether ANA are simple bystanders or if they can have a role in the pathophysiology of the disease. One might think that the nuclear nature of their targets prevents any accessibility to autoantibodies. Nevertheless, recent data suggest that ANA could be pathogenic or at least contribute to the perennation of the disease. We review here first the indirect clues of the contribution of ANA to SSc: they are associated to the disease subtypes, they may precede disease onset, their titer correlates with disease activity and severity, there is an association between molecular subsets, and some patients can respond to B-cell targeting therapy. Then, we describe in a second part the mechanisms of ANA production in SSc from individual genetic background to post-transcriptional modifications of neoantigens. Finally, we elaborate on the potential mechanisms of pathogenicity: ANA could be pathogenic through immune-complex-mediated mechanisms; other processes potentially involve molecular mimicry and ANA penetration into the target cell, with a focus on anti-topoisomerase-I antibodies, which are the most probable candidate to play a role in the pathophysiology of SSc. Finally, we outline some technical and conceptual ways to improve our understanding in this field.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

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

Submission date :

2024-01-12T01:03:27Z
2024-02-28T10:05:01Z

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