Systemic sclerosis (scleroderma) is a chronic, connective tissue disease affecting skin, musculoscleletal system, cardiovascular system, the lungs, kidneys and gastrointestinal tract. Early detection and management of scleroderma-related organ involvement is of key importance for improving health-related quality of life and reduction of mortality in patients with systemic sclerosis. In view of significant heterogeneity in the clinical manifestations and progression of the disease, management of patient with systemic sclerosis should be designed based on the risk of and/or the presence of particular organ involvement (organ-specific therapy). Since there is only little evidence concerning efficacy of therapies used for treating scleroderma-related organ involvement, experience of a team providing treatment and clinical care has a pivotal role in proper management of patients with systemic sclerosis.