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Abstract
Systemic sclerosis, also called scleroderma, is an immune-mediated rheumatic disease
that is characterised by fibrosis of the skin and internal organs and vasculopathy.
Although systemic sclerosis is uncommon, it has a high morbidity and mortality. Improved
understanding of systemic sclerosis has allowed better management of the disease,
including improved classification and more systematic assessment and follow-up. Additionally,
treatments for specific complications have emerged and a growing evidence base supports
the use of immune suppression for the treatment of skin and lung fibrosis. Some manifestations
of the disease, such as scleroderma renal crisis, pulmonary arterial hypertension,
digital ulceration, and gastro-oesophageal reflux, are now treatable. However, the
burden of non-lethal complications associated with systemic sclerosis is substantial
and is likely to become more of a challenge. Here, we review the clinical features
of systemic sclerosis and describe the best practice approaches for its management.
Furthermore, we identify future areas for development.