Targeting very early systemic sclerosis: a case-based review

It is unknown whether treatment in very early/early systemic sclerosis (SSc) can affect long-term outcomes. A case-based review was conducted (i) to assess the effect of rituximab (RTX) in very early SSc and (ii) to explore how many clinical trials in SSc targeted early disease and whether treatment of these patients led to better clinical outcomes. We identified cases of very early SSc from our department and performed a search in MEDLINE and Scopus databases for clinical trials in SSc during 2005–2018. Two cases are reported where RTX was administered within 24 months from the appearance of Raynaud’s. In the first case, there was an improvement in interstitial lung disease as indicated by the improvement in pulmonary function tests and the regression of changes in high-resolution chest computed tomography. In the second case, a good clinical response in skin fibrosis was observed. The review revealed the following: (i) only one-third of the studies were specifically designed to target early disease, (ii) there is confusion related to disease duration definition across SSc clinical trials but an obvious trend towards improvement was evident during the past years, (iii) the question of whether early implementation of therapy may lead to better clinical outcomes cannot be definitely answered based on existing data and (iv) there is still a very low level of incorporation of the new classification criteria in SSc trials. This review suggests that there may be a window of opportunity in SSc and highlights the need for clinical trials targeting very early/early disease.