Systemic sclerosis—challenges for clinical practice

Abstract

The management of patients with systemic sclerosis (SSc) can be challenging because disease-associated damage is often difficult to reverse and curative therapies are not yet available. Early identification and appropriate monitoring of patients with SSc is, therefore, critical so that active disease can be controlled and tissue damage prevented or delayed. However, early diagnosis of SSc is often difficult because the early clinical stages of the disease can be very similar to that of other autoimmune conditions. Screening for major organ manifestations of SSc, particularly interstitial lung disease, pulmonary hypertension, renal involvement and cardiac disease is a priority because involvement of these organs is associated with shorter life expectancies and early intervention might prevent disease progression. The prevention and management of digital ischaemia is also important as appropriate therapy often prevents substantial morbidity and functional loss. Treating gastrointestinal dysmotility can usually be managed using proton pump inhibitors and promotility agents, although in severe cases total parenteral nutrition is required. Calcinosis in patients with SSc is another common challenge that requires appropriate disease management and pain control. Each of these topics, which are relevant to both physicians and patients with SSc, are reviewed in further detail herein.