Emerging targets of disease-modifying therapy for systemic sclerosis

Elizabeth R. Volkmann, John Varga*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

84 Scopus citations


Systemic sclerosis (SSc) has the highest cause-specific mortality of all the connective tissue diseases, and the aetiology of this complex and heterogeneous condition remains an enigma. Current disease-modifying therapies for SSc predominantly target inflammatory and vascular pathways but have variable and unpredictable clinical efficacy, and none is curative. Moreover, many of these therapies possess undesirable safety profiles and have no appreciable effect on long-term mortality. This Review describes the most promising of the existing therapeutic targets for SSc and places them in the context of our evolving understanding of the pathophysiology of this disease. As well as taking an in-depth look at the immune, inflammatory, vascular and fibrotic pathways implicated in the pathogenesis of SSc, this Review discusses emerging treatment targets and therapeutic strategies. The article concludes with an overview of important unanswered questions in SSc research that might inform the design of future studies of treatments aimed at modifying the course of this disease.

Original languageEnglish (US)
Pages (from-to)208-224
Number of pages17
JournalNature Reviews Rheumatology
Issue number4
StatePublished - Apr 1 2019

ASJC Scopus subject areas

  • Rheumatology


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