Management of systemic sclerosis

Abstract

Appropriate management of systemic sclerosis (SSc) requires accurate disease subsetting, staging of the disease within each subset and risk stratification for major organ-based complications, based upon clinical features and serology All patients with SSc should be screened for major complications to facilitate early intervention Hypertensive renal crisis can occur in any patients with SSc; angiotensin-converting enzyme inhibitors should be instituted as early as possible in these cases Significant reduction in transfer factor on lung function tests may reflect either interstitial lung disease or pulmonary hypertension (PAH). Doppler echocardiography and high-resolution computed tomography of the chest are indicated PAH should be confirmed by right heart catheterisation before considering advanced therapy for symptomatic cases.