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Review
. 2020 Oct;7(Suppl 3):S222-S227.
doi: 10.5152/eurjrheum.2020.19116. Epub 2020 Jul 6.

Systemic sclerosis: To subset or not to subset, that is the question

Sindhu R Johnson  1   2 , Frank van den Hoogen  3 , Keshini Devakandan  1 , Marco Matucci-Cerinic  4 , Janet E Pope  5
Affiliations
Review

Systemic sclerosis: To subset or not to subset, that is the question

Sindhu R Johnson et al. Eur J Rheumatol. 2020 Oct.

Abstract

Systemic sclerosis (SSc) is a heterogeneous disease with variability in autoantibody profiles, skin and internal organ involvement, disease trajectory, and survival. The ability to identify more homogeneous subsets of SSc patients has informed patient care and been an essential aspect of SSc research. In this article, the historic evolution of subsetting systems in SSc are described including clinically based SSc subsetting systems, their utility, strengths, and limitations. There is a shifting paradigm of SSc subsets, including biologic classification of SSc subsets and fully data-driven approaches to SSc subset classification, taking into consideration the needs of the SSc global community in the modern era and the ability to prognosticate patients with SSc.

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Conflict of interest statement

Conflict of Interest: S.R.J. is supported by the Ontario Ministry of Research and Innovation, Ministry of Economic Development, Trade and Employment Early Researcher Award, the Canadian Institutes of Health Research New Investigator Award and the Oscar and Eleanor Markovitz Fund for Scleroderma Research of the Arthritis Research Foundation. She is also supported by the Gurmej Kaur Dhanda Scleroderma Research Award. The other authors have no conflict of interest to declare.

References

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