Nevskaya, T;
Pope, JE;
Turk, MA;
Shu, J;
Marquardt, A;
van den Hoogen, F;
Khanna, D;
... Johnson, SR; + view all
(2021)
Systematic analysis of the literature in search of defining systemic sclerosis subsets.
The Journal of Rheumatology
, 48
(11)
pp. 1698-1717.
10.3899/jrheum.201594.
(In press).
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Abstract
OBJECTIVE: Systemic sclerosis (SSc) is a multisystem disease with heterogeneity in presentation and prognosis. An international collaboration to develop new SSc subset criteria is underway. Our objectives were to identify systems of SSc subset classification and synthesize novel concepts to inform development of new criteria. METHODS: Medline, Cochrane MEDLINE, the Cumulative Index to Nursing and Allied Health Literature, EMBASE, and Web of Science were searched from their inceptions to December 2019 for studies related to SSc subclassification, limited to humans and without language or sample size restrictions. RESULTS: Of 5686 citations, 102 studies reported original data on SSc subsets. Subset classification systems relied on extent of skin involvement and/or SSc-specific autoantibodies (n = 61), nailfold capillary patterns (n = 29), and molecular, genomic, and cellular patterns (n = 12). While some systems of subset classification confer prognostic value for clinical phenotype, severity, and mortality, only subsetting by gene expression signatures in tissue samples has been associated with response to therapy. CONCLUSION: Subsetting on extent of skin involvement remains important. Novel disease attributes including SSc-specific autoantibodies, nailfold capillary patterns, and tissue gene expression signatures have been proposed as innovative means of SSc subsetting.
| Type: | Article |
|---|---|
| Title: | Systematic analysis of the literature in search of defining systemic sclerosis subsets |
| Location: | Canada |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3899/jrheum.201594 |
| Publisher version: | https://doi.org/10.3899/jrheum.201594 |
| Language: | English |
| Additional information: | Copyright © 2021 by the Journal of Rheumatology This is an Open Access article, which permits use, distribution, and reproduction, without modification, provided the original article is correctly cited and is not used for commercial purposes. |
| Keywords: | Autoimmune diseases, scleroderma, systemic sclerosis |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10128850 |
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