Systemic sclerosis (SSc) is a multisystem and complex disease, with different classifications as well as different degrees of pulmonary involvement. SSc patients, and particularly those with a diffuse disease, are at increased risk of developing an interstitial lung disease (ILD), pulmonary arterial hypertension (PAH) or lung involvement associated with pulmonary hypertension (ILD-PH) which, if untreated, may lead to right ventricular failure and premature death [1]. The pulmonary involvement, if present, is responsible for a reduced exercise capacity and represents the leading cause of SSc-related deaths.
