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Feature Article

Systemic sclerosis: a step-by-step guide to a difficult diagnosis

Joanna Tieu, Susanna Proudman

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Abstract

Systemic sclerosis is an uncommon condition but the associated complications can have severe adverse effects on a patient’s quality of life and survival. A multimodal, multidisciplinary approach to management of disease manifestations is essential.

Key Points

  • Systemic sclerosis (SSc), or scleroderma, is a multisystem autoimmune disease characterised by vasculopathy and fibrosis.
  • Adult-onset Raynaud’s phenomenon or Raynaud’s phenomenon complicated by digital ulceration or infarction should prompt consideration of connective tissue disorders including SSc.
  • Early identification of severe, life-threatening organ manifestations can result in improved quality of life and survival.
  • Management of patients with SSc is multifaceted and dependent on individual disease manifestations.

    Picture credit: © Phototake/Bodell Communications/Diomedia.com

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