Pathogenic role of anti‐nuclear autoantibodies in systemic sclerosis: Insights from other rheumatic diseases

Summary Systemic sclerosis (SSc) is a severe autoimmune disease characterized by vasculopathy, fibrosis, and dysregulated immunity, with hallmark autoantibodies targeting nuclear antigens such as centromere protein (ACA) and topoisomerase I (ATA). These autoantibodies are highly prevalent and diseas...

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Bibliographic Details
Published in:Immunological reviews 2024-11, Vol.328 (1), p.265-282
Main Authors: Oostveen, Wieke M., Huizinga, Tom W. J., Fehres, Cynthia M.
Format: Article
Language:English
Subjects:
Animals
Anti-Citrullinated Protein Antibodies - immunology
Anti-Citrullinated Protein Antibodies - metabolism
Antibodies in Pathology
Antibodies, Antinuclear - immunology
Antigens
anti‐centromere antibodies
anti‐topoisomerase I antibodies
Autoantibodies
Autoimmune diseases
B-lymphocytes
Biomarkers
centromeres
Citrulline
disease diagnosis
DNA topoisomerase
DNA Topoisomerases, Type I - immunology
DNA Topoisomerases, Type I - metabolism
Fibrosis
Glycosylation
Humans
immunity
Invited Review
Pathogenesis
Proteins
Rheumatic diseases
Rheumatic Diseases - diagnosis
Rheumatic Diseases - immunology
Rheumatoid arthritis
Scleroderma, Systemic - immunology
sclerosis
Systemic sclerosis
therapeutics
Vascular diseases
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Summary:Summary Systemic sclerosis (SSc) is a severe autoimmune disease characterized by vasculopathy, fibrosis, and dysregulated immunity, with hallmark autoantibodies targeting nuclear antigens such as centromere protein (ACA) and topoisomerase I (ATA). These autoantibodies are highly prevalent and disease‐specific, rarely coexisting, thus serving as crucial biomarkers for SSc diagnosis. Despite their diagnostic value, their roles in SSc pathogenesis remain unclear. This review summarizes current literature on ACA and ATA in SSc, comparing them to autoantibodies in other rheumatic diseases to elucidate their potential pathogenic roles. Similarities are drawn with anti‐citrullinated protein antibodies (ACPA) in rheumatoid arthritis, particularly regarding disease specificity and minimal pathogenic impact of antigen binding. In addition, differences between ANA and ACPA in therapeutic responses and Fab glycosylation patterns are reviewed. While ACA and ATA are valuable for disease stratification and monitoring activity, understanding their origins and the associated B cell responses is critical for advancing therapeutic strategies for SSc.
ISSN:0105-2896
1600-065X
1600-065X
DOI:10.1111/imr.13390