Systemic sclerosis: clinical features and management

Systemic sclerosis (SSc) is an important autoimmune rheumatic disease within the scleroderma spectrum of disorders. It is the prototypic multi-system fibrotic disease with important complications that also result from vasculopathy and inflammation. There have been substantial recent advances in understanding the pathogenic mechanisms underlying SSc and this has facilitated more logical treatment approaches. While it remains the rheumatic disease with the highest case-specific mortality, management of organ-specific complications has now advanced, with emerging clinical evidence supporting the use of immunosuppression in SSc lung fibrosis, and important advances in the management of pulmonary arterial hypertension. Systemic sclerosis should not be considered untreatable, and patient and physician education about potential treatments is an important aspect of management. However, therapies must be carefully matched to disease subset and to the stage of disease.