Systemic sclerosis is a multisystem connective tissue disease which can present to a broad range of medical specialities. It carries significant morbidity and mortality, but substantial advances have been made in recent years. Recent advances include autologous haematopoietic stem cell transplantation for early diffuse disease (for highly selected patients), the increasing use of phosphodiesterase type 5 inhibitors and of bosentan for digital ulceration, a better understanding of the use of different immunosuppressant therapies for interstitial lung disease (and which patients are likely to benefit from antifibrotic therapy), and the increasing early use of combination therapies for pulmonary arterial hypertension (PAH). Ongoing clinical trials investigating new therapeutic approaches should lead to further advances in the next 10 years. This review provides a broad overview of the condition with a focus on recent progress in specific areas: early diffuse cutaneous disease, digital vasculopathy, interstitial lung disease, and PAH.