Scleroderma

Abstract

Systemic sclerosis (SSc) is a complex, autoimmune rheumatic disease that can result in high morbidity and mortality due to tissue fibrosis and vascular damage. In the past ten years, clinical trials in SSc were largely limited by a disappointing end point, the modified Rodnan skin score, which can improve over time, even on placebo. With the development of a composite end point, the CRISS (Composite Response Index for Systemic Sclerosis), there has been an advent of phase 2/3 clinical trials to target disease-specific organ manifestations. The most significant strides gained in the treatment of SSc has been in the cardiopulmonary manifestations of the disease—specifically SSc-associated interstitial lung disease (ILD) and pulmonary arterial hypertension. In fact, in the past two years, in 2020 and 2021, the FDA approved two drugs to treat SSc-associated ILD, the first ever approved in the history of the treatment of SSc. With these newly approved drugs, much hope is placed in future clinical trials to also treat early diffuse cutaneous SSc.