• interstitial fibrosis

Interstitial lung disease (ILD) is a frequent organ manifestation in systemic sclerosis (SSc) and is associated with high morbidity and mortality.1 The severity and disease course vary widely from mild and stable disease to severe and rapidly progressing.2 Early detection of ILD and prediction of disease progression are key in the management of SSc-ILD, as any ILD in SSc is associated with reduced long-term outcome especially in case of disease progression.1 There has been substantial progress in the treatment of this devastating condition, but allocation of the right treatment at the right time for any given individual with SSc-ILD remains challenging. While several risk factors for ILD progression in SSc have been identified in the past, the predictive ability of single factors is very moderate.3 Combination of different risk factors such as early disease, increased inflammatory markers, recent skin progression and the diffuse cutaneous SSc subset have been shown to improve the identification of patients at risk of progression.3 However, a high unmet need still exists to specifically identify the individual patient with SSc-ILD who is at risk of progressive disease, to initiate treatment early, to prevent progression and to avoid irreversible …