2022
DOI: 10.3389/fimmu.2022.930970
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Can Antinuclear Antibodies Have a Pathogenic Role in Systemic Sclerosis?

Abstract: 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 … Show more

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Cited by 26 publications
(18 citation statements)
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“…Positive ANA have been detected in up to 50% of patients with acute SARS-CoV-2 infection ( 19 , 20 ) but, conversely, long-term data are limited and contradictory ( 21 , 22 ). ANAs have been suggested to play a pathogenic role in disease through different mechanisms, including the formation and deposition of immune complexes containing ANA and nuclear autoantigen, molecular mimicry and direct interaction on target cells or penetration into cells ( 23 ). It remains to be determined whether they might potentially contribute to the severity and persistence of symptoms associated with COVID-19.…”
Section: Discussionmentioning
confidence: 99%
“…Positive ANA have been detected in up to 50% of patients with acute SARS-CoV-2 infection ( 19 , 20 ) but, conversely, long-term data are limited and contradictory ( 21 , 22 ). ANAs have been suggested to play a pathogenic role in disease through different mechanisms, including the formation and deposition of immune complexes containing ANA and nuclear autoantigen, molecular mimicry and direct interaction on target cells or penetration into cells ( 23 ). It remains to be determined whether they might potentially contribute to the severity and persistence of symptoms associated with COVID-19.…”
Section: Discussionmentioning
confidence: 99%
“…In SSc, apoptotic blebs of endothelial cells and Topo-1 release from by apoptotic blebs are described. 8,51,52 In addition, the release of neutrophil extracellular traps (NETs), also called NETosis, has also been highlighted in SLE as a contributor to autoimmunity and a source for autoantibodies. Recently, Didier et al could demonstrate evidence for NETosis also in SSc.…”
Section: Discussionmentioning
confidence: 99%
“…10 Newer data suggest that antibodies could be pathogenic or at least contribute to the perennation of the disease. 8,11,12 In addition, they have been established as strong predictors of disease outcome, of certain organ complications and therapeutic response. [13][14][15] For example, Topo-1 and RP3 are more specific for dcSSc than ACA.…”
Section: Introductionmentioning
confidence: 99%
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“…Many patients present with specific anti-centromeres antibodies (ACA), antibodies against topoisomerase 1 (Topo-1, also known as Scl-70) and/or RNA polymerase III (RP3) antibodies (5). The mechanisms underlying the development of these distinct antibodies in SSc are widely unclear, but accumulating data suggest a specific genetic background in combination with environmental and stochastic factors, as well as properties of the antigens themselves are key in antigen selection and antibody production (6)(7)(8)(9). Moreover, the pathogenetic role of these autoantibodies in SSc is still a subject of ongoing research (10).…”
Section: Introductionmentioning
confidence: 99%