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  • Review Article
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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.

Key Points

  • Screening and identification of risk factors for internal organ manifestations of systemic sclerosis (SSc) is important and should be done soon after diagnosis and regularly thereafter

  • Autoantibody testing can be used to identify patients who are at increased risk of specific scleroderma-related complications; these patients should be closely monitored for early detection of such complications

  • Interstitial lung disease and pulmonary hypertension are serious and often deadly complications of scleroderma; patients with these complications should receive aggressive treatment early in the disease process

  • In patients with scleroderma, gastrointestinal disease can be severe and targeted intervention with gastrointestinal antacids is usually required

  • Promotility agents can also improve symptoms of gastrointestinal disease but in severe cases, total parenteral nutrition might be required

  • Scleroderma is a complex multi-organ disease and the management of affected patients often requires coordinated efforts with experienced specialists in other fields

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Figure 1: Prototypical ischaemic digital fingertip ulcerations in an adult patient with systemic sclerosis.

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Acknowledgements

The authors would like to thank the Division of Rheumatology at Johns Hopkins and the Johns Hopkins Scleroderma Center for their ongoing support.

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Both authors researched the data for the article, wrote the article and reviewed and/or edited the manuscript before submission.

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Correspondence to Laura K. Hummers.

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McMahan, Z., Hummers, L. Systemic sclerosis—challenges for clinical practice. Nat Rev Rheumatol 9, 90–100 (2013). https://doi.org/10.1038/nrrheum.2012.191

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