The development of treatments for systemic sclerosis has historically been hampered by the clinical heterogeneity of the disease and limited understanding of its pathogenesis. Encouragingly, advances including the identification of important molecular targets and improvements in clinical trial design have now greatly increased the number of investigative therapies.
This is a preview of subscription content, access via your institution
Access options
Access Nature and 54 other Nature Portfolio journals
Get Nature+, our best-value online-access subscription
$29.99 / 30 days
cancel any time
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
Hao, Y. et al. Early mortality in a multinational systemic sclerosis inception cohort. Arthritis Rheum. 69, 1067–1077 (2017).
Campochiaro, C. & Allanore, Y. An update on targeted therapies in systemic sclerosis based on a systematic review from the last 3 years. Arthritis Res. Ther. 23, 155 (2021).
Chung, M. P. & Chung, L. Drugs in phase I and phase II clinical trials for systemic sclerosis. Expert Opin. Investig. Drugs. 29, 349–362 (2020).
Distler, O. et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N. Engl. J. Med. 380, 2518–2528 (2019).
Khanna, D. et al. Tocilizumab in systemic sclerosis: a randomized, double-blind, placebo-controlled, phase 3 trial. Lancet Respir. Med. 8, 963–974 (2020).
Khanna et al. Abatacept in early diffuse cutaneous systemic sclerosis: results of a phase II investigator-initiated, multicenter, double-blind, randomized, placebo-controlled trial. Arthritis Rheumatol. 72, 125–136 (2020).
Maurer, B. et al. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database. Ann. Rheum. Dis. 74, 1124–1131 (2015).
Domsic, R. T., Rodriguez-Reyna, T., Lucas, M., Fertig, N. & Medsger, Jr T. A. Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann. Rheum. Dis. 70, 104–109 (2011).
Roofeh, D. et al. Tocilizumab prevents progression of early systemic sclerosis associated interstitial lung disease. Arthritis Rheumatol. 73, 1301–1310 (2021).
Nagaraja, V. et al. Current and future outlook on disease modification and defining low disease activity in systemic sclerosis. Arthritis Rheumatol. 72, 1049–1058 (2020).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
L.C. declares that she has served as a consultant and/or advisor and has received grant funding from Boerhinger Ingelheim, has served on the Steering Committee and acted as a consultant and/or advisor for Eicos Sciences, has served as a consultant and/or advisor for Mitsubishi Tanabe and Genentech and was a member of the Data Safety Monitoring Board for Reata. Y.K. declares no competing interests.
Rights and permissions
About this article
Cite this article
Kawano, Y., Chung, L. Promise and challenge of systemic sclerosis therapies. Nat Rev Rheumatol 17, 581–582 (2021). https://doi.org/10.1038/s41584-021-00678-z
Published:
Issue Date:
DOI: https://doi.org/10.1038/s41584-021-00678-z