The pathogenic process of systemic sclerosis targets the skin and internal organs, and involves sequential or concomitant abnormalities in blood vessel function, immunity and subsequent fibroblast function. These characteristics are disease specific and may partly explain the unresolved therapeutic strategies that must take into account not only these various biological abnormalities, but also the complexities of the various abnormalities throughout the duration of the disease. However, recent epidemiological data have revealed a decrease in excess mortality, which may be mostly due to the use of cardiovascular drugs. This article deals with present and possible future therapeutic options.