Systemic sclerosis-associated interstitial lung disease - proposed recommendations for future randomised clinical trials

摘要

Pulmonary disease is the leading cause of morbidity and mortality in patients with systemic sclerosis (SSc). Recent well-designed trials in SSc-associated interstitial lung disease (SSc-ILD) have provided important insights regarding outcome measures and trial design. Recent investigations into the pathogen-esis of SSc-ILD have led to a renewed interest in assessing targeted therapies in SSc-ILD. With this in mind, we propose recommendations for the design of future SSc-ILD studies in this review.Pulmonary disease is the leading cause of morbidity and mortality in patients with systemic sclerosis (SSc) (1). Lung involvement in SSc can be separated into two distinct entities: pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) although many patient have elements of both. Recent well-designed trials in SSc-ILD have provided useful information regarding outcome measures and trial design. This, coupled with active investigation into the pathogenesis of SSc-ILD, has led to an interest in assessing targeted therapies in SSc-ILD. Reliable trial design is a requisite to assessment of these potential therapies. With that in mind, we propose recommendations for the design of future SSc-ILD studies (Table I).