Systemic sclerosis (scleroderma) is a rheumatologic disease characterised not only by inflammation/autoimmunity, but also by tissue fibrosis and vascular lesions. The therapeutic approach to patients is dictated by the organ involvement and includes treatment of vascular and fibrotic disease features beyond mere immunosuppression. Fibrotic features in particular, are still inadequately treated, whereas many drugs have been tested for vascular complications within recent years. In this review, the currently available treatment options for this rare disease are presented. Therapy options in systemic sclerosis have changed over the past 10 years and this trend will also continue in the future.