An update on autoantibodies in scleroderma
- PMID: 30148799
- PMCID: PMC6546089
- DOI: 10.1097/BOR.0000000000000550
An update on autoantibodies in scleroderma
Abstract
Purpose of review: New research continues to provide important insights into the utility of antibody specificities. This review provides an update of recent findings, and the important insights they provide into disease mechanism.
Recent findings: A growing number of autoantibodies have been discovered in scleroderma patients with unique clinical associations. A subgroup of these antibodies may have functional consequences and contribute to disease pathogenesis, driving the vascular and fibrotic phenotype. Recent research into the relationship between malignancy and scleroderma onset provides important new insights into disease mechanism, and highlights the utility of autoantibodies as unique research probes.
Summary: Continued advances in the study of scleroderma antibody specificities has led to important insights into disease pathogenesis and clinical subgrouping. These advances include newly described specificities, functional antibodies and an emerging understanding of the cancer-scleroderma relationship.
References
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Seminal paper providing evidence that scleroderma in a subgroup of RNApol3-positive patients results from the immune system’s response to the cancer. Studying cancers in scleroderma patients, genetic alterations were discovered in the POLR3A locus of cancers in RNApol3-positive patients but not RNApol3 negative, suggesting the autoimmune disease may be initiated by exposure to foreign antigens that cross-reactive with endogenous molecules.
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- Lazzaroni M-G, Cavazzana I, Colombo E, Dobrota R, Hernandez J, Hesselstrand R, … Airò P (2017). Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening. The Journal of Rheumatology, 44(5), 639–647. 10.3899/jrheum.160817 - DOI - PubMed
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