Systemic sclerosis: clinical features and management

There have been substantial recent advances in understanding the pathogenic mechanisms underlying systemic sclerosis (SSc) and this has facilitated more logical treatment approaches. There have been positive clinical trials for immunosuppression in SSc lung fibrosis, in digital vasculopathy and in pulmonary arterial hypertension. Advances in management have occurred for many of the organ-based complications of SSc. However, it remains a challenging and clinically diverse disease with a high mortality and substantial morbidity. Systemic sclerosis forms an important part of the scleroderma spectrum of disorders that includes localized forms of scleroderma and overlap syndromes that include clinical features of other autoimmune rheumatic diseases. Systemic sclerosis should not be considered untreatable and patient and physician education about potential treatments is an important aspect of management. Therapies must however be carefully matched to disease subset and to the stage of disease.