Systemic sclerosis is a challenging disease in terms of early risk assessment and the implications for therapy. The current article draws on a literature analysis and on personal experience of over 450 patients with systemic sclerosis and related illnesses, describing an attempt at a risk-adapted treatment strategy for patients with systemic sclerosis, and is intended to be used as a discussion paper. A major point of emphasis is on novel therapeutic options for patients who are refractory to current treatment strategies and who are at a high risk of serious complications or a limited prognosis. The paper also highlights possible future treatment options, including vasoactive, cellular and immune suppressant treatments.