Systemic sclerosis has high mortality and morbidity and is diverse in clinical manifestations. In approaching management it is important to consider baseline assessment that must include confirmation of diagnosis, ascertainment of any features of overlap connective tissue disease and determining the subset of disease (limited or diffuse) based upon the extent of skin sclerosis. Initial assessment should also include definition of the presence of any hallmark ANA reactivity that may predict the development or presence of internal organ disease. Baseline assessment of cardiac, pulmonary and renal function together with general evaluation and determination of modified Rodnan skin score is undertaken. Treatment depends on the stage and subset and pattern of disease and generally follows the EULAR-EUSTAR recommendations that suggest options for skin, lung and other internal organ disease. All patients will need treatment of secondary Raynaud's phenomenon and gastro-oesophageal reflux symptoms. Raynaud's phenomenon can be treated by oral vasodilators or in severe cases with parenteral prostacyclin and other strategies. Skin disease is generally treated with immunosuppression such as MMF or MTX and lung fibrosis is assessed depending on a well validated staging system, This is more than 20% lung parenchyma is judged affected then immunosuppression is used, often with intravenous cyclophosphamide. Other agents may be considered, especially for overlap disease with myositis and arthritis. High does corticosteroids may increase risk of renal crisis and are avoided if possible although low doses less than 10mg prednisolone per day may give symptomatic benefit for inflammatory symptoms and skin itching. If pulmonary hypertension (PH) is suspected then this must be investigated, often including right heart catheterisation and management is in line with other types of PH, including targeted therapies for pre-capillary pulmonary arterial hypertension (PAH). Outcomes have improved over the past two decades but vigilant follow up and attention to non-lethal complications that impact on quality of life is essential. These topics will be reviewed.
Disclosure of Interest None declared