Therapeutic options for systemic sclerosis.

ABSTRACT

Systemic sclerosis is a uncommon connective tissue disorder characterized by vascular damage, immune cell activation and fibrogenesis. Each of these components may respond to different therapies. Therefore, a combination strategy treating all three processes is more likely to control the disease than single agent therapy. Clinical trials have gone a long way towards defining the therapy of scleroderma and many drugs previously used for scleroderma have been critically assessed. Angiotensin blockade is effective in treating as well as preventing scleroderma renal crisis. The 9-year cumulative survival has improved from 38% to 68% after the introduction of angiotensin blockade. There is definitive evidence supporting the use of cyclophosphamide in systemic sclerosis associated alveolitis. Newer molecules aimed at various cytokines are being tried. The therapy for systemic sclerosis is far from perfect at present. But, individualization of the treatment with respect to stage and subset of disease as well as organ involvement can eventually result in rational, effective management.