Systemic Sclerosis
The Lancet Rheumatology
Published: February 28, 2020
Systemic sclerosis (SSc) remains the deadliest of the rheumatic diseases, despite improvements in the survival of these patients in recent decades. Clinical management of patients with SSc is challenging, and treatment options are limited. Until the 2019 approval of nintedanib for SSc-associated interstitial lung disease (ILD), there had been no major breakthroughs in organ-specific treatments since the introduction of the ACE inhibitor captopril for scleroderma renal crisis, nearly four decades ago. And no effective treatment exists that combats the disease across organ systems. Clinical heterogeneity among patients with SSc presents a formidable challenge, not only to the understanding of disease pathophysiology, but also the therapeutic management of patients and the design of clinical trials. Indeed, disappointments have outnumbered successes in SSc trials, with many trials of potentially promising agents failing to meet their primary endpoints. This joint Series in The Lancet Rheumatology and The Lancet Respiratory Medicine explores the latest advances in SSc and SSc-associated ILD, with a focus on novel and innovative approaches to trial design and patient stratification, and a discussion of new and potentially more robust outcome measures in SSc—all of which will be essential to achieve progress toward new therapies for patients with this disabling disease.