Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by widespread vascular injury and progressive fibrosis of the skin and internal organs. The involvement of internal organs results in significant morbidity and mortality of SSc patients with cardiopulmonary involvement being the leading cause of SSc‑related deaths. The management of SSc patients remains a challenge because therapeutic options are rather limited and no therapy has definitively shown a disease‑modifying effect. A significant progress that has recently been made in the understanding of the SSc pathogenesis contributed to the introduction of new therapeutic options. Preliminary clinical studies have yielded promising results for mycophenolate mofetil, anti‑CD20 antibodies, and stem‑cell in the treatment of SSc. Multicenter cohort studies help understand the natural history of SSc, which leads to improvement in the care of SSc patients. The major objective of those studies is to establish the screening strategies for early diagnosis and, subsequently, to introduce appropriate management concerning specific organ involvement in SSc as well as to formulate specific treatment recommendations.