Systemic sclerosis is an immune-mediated disease characterized by a chronic progressive course. The complex and diverse clinical features seen in patients require case-by-case approach and cooperation of multiple specialists both at the stage of diagnosis and treatment. Critical factors in systemic sclerosis include early diagnosis, assessment of internal organ involvement, identification of patients at potential risk of organ complications, assessment of disease dynamics and activity, and implementation of optimal therapy. Treatment decisions should be made individually for each patient after careful consideration of such aspects as the severity of skin lesions, duration and activity of the disease, manifestations and possible internal organ involvement. On account of potential organ dysfunctions, patients require a multidisciplinary therapeutic approach and coordinated treatment by experienced specialists or in specialist medical centres. Part 2 presents current guidelines for the treatment of systemic sclerosis.