Increasing knowledge about the rare disease systemic sclerosis and improved diagnostic methods in the course of recent decades has led to the possibility of diagnosing systemic sclerosis at earlier disease stages. However, earlier diagnosis has an impact on routine clinical care of affected patients, and rheumatologists need to know about early symptoms, their diagnosis, and clinical management. In this review, the therapeutic management of early disease stages is described. In particular, we focus on diagnostic tools which should be included in a "basic assessment" of systemic sclerosis and discuss the diagnosis and treatment options of early symptoms such as Raynaud phenomenon, puffy fingers and hand edema, digital ulcers, calcinosis cutis, and cardiopulmonary, renal, and gastrointestinal involvement. Finally, the options of early immunosuppressive treatment and autologous stem cell transplantation for patients with rapid progressive and severe disease are reviewed.