The clinical manifestations of systemic sclerosis (scleroderma, SSc) are characterized by three prominent features: autoimmunity/inflammation, vascular lesions (vasculopathy) and (organ) fibrosis. Drugs and other therapies are now available for each of these features. However, due to the low prevalence and high variation in clinical signs and symptoms of the disease, there are only few high quality clinical trials. The EULAR Scleroderma Trials and Research Group (EUSTAR, a subgroup of the European League Against Rheumatism, EULAR) has therefore started to assess the therapies available today and make recommendations. The present article discusses treatment options in systemic sclerosis beyond these recommendations. It is to be expected that the establishment of national and international networks for systemic sclerosis research (e.g. the German Network for Systemic Sclerosis, DNSS, and EUSTAR) will raise the standards of evidence-based therapy for systemic sclerosis in the future by analyzing large data sets and performing clinical trials.