Practical approach to the therapy of systemic sclerosis

Systemic sclerosis (SSc) is characterised by vascular abnormalities, fibrosis, inflammatory changes, and atrophy affecting the skin and several internal organs. The course of the disease is highly variable from patient to patient, and the different organ manifestations may show a different stage of activity and damage. More extensive skin involvement coincides with more severe internal organ manifestation(s) resulting in an unfavourable outcome of disease. Subset classification and organ system involvements should be carefully determined before introducing the therapy. Prognostic evaluation, including the staging of disease severity, damage, activity and the definition of longitudinal disease behaviour of the organ manifestations (by serial investigations) is also crucial. Theoretically the treatment should focus on both the early reduction of inflammatory phenomena and influence of vascular symptoms to prevent the development of tissue fibrosis and atrophy. Major internal organ manifestations including gastro-oesophageal reflux, scleroderma renal involvement, different cardio-respiratory disorders should also be carefully looked for, and early signs of internal organ involvements should be recognized and treated. There is no disease modifying drug therapy of proven efficacy in SSc but an individualized, well designed therapy may be remarkably efficient in terms of improving the prognosis and quality of life of patients. Education of patients is also compelling.