Raynaud phenomenon and digital ulcers in systemic sclerosis

Michael Hughes, Yannick Allanore, Lorinda Chung, John D. Pauling, Christopher P. Denton, Marco Matucci-Cerinic

Research output: Contribution to journalReview article

6 Citations (Scopus)
4 Downloads (Pure)

Abstract

Raynaud phenomenon is a symptom complex caused by impaired digital perfusion and can occur as a primary phenomenon or secondary to a wide range of underlying causes. Raynaud phenomenon occurs in virtually all patients with systemic sclerosis (SSc) and is often the earliest clinical manifestation to occur. Careful assessment is required in patients with Raynaud phenomenon to avoid missing secondary causes such as SSc. Digital ulcers are a painful and disabling visible manifestation of digital vascular injury in patients with SSc. Progress has been made in the classification and assessment of digital ulcers and in understanding ulcer pathogenesis, and there are a wide range of treatments available to both prevent and heal digital ulcers, some of which are also used in Raynaud phenomenon management. In this Review, the assessment of patients with Raynaud phenomenon is discussed, including ‘red flags’ that are suggestive of SSc. The pathogenesis, classification and assessment of SSc-associated digital ulcers are also covered, alongside an overview of management approaches for SSc-associated Raynaud phenomenon and digital ulcers. Finally, unmet needs are discussed and the concept of a unified vascular phenotype in which therapies that affect the vasculature to support disease modification strategies is introduced.

Original languageEnglish
Pages (from-to)208-221
Number of pages14
JournalNature Reviews Rheumatology
Volume16
Issue number4
Early online date25 Feb 2020
DOIs
Publication statusPublished - 30 Apr 2020

Keywords

  • Fingers/blood supply
  • Humans
  • Raynaud Disease/complications
  • Scleroderma, Systemic/complications
  • Skin Ulcer/diagnosis

Cite this

Hughes, M., Allanore, Y., Chung, L., Pauling, J. D., Denton, C. P., & Matucci-Cerinic, M. (2020). Raynaud phenomenon and digital ulcers in systemic sclerosis. Nature Reviews Rheumatology, 16(4), 208-221. https://doi.org/10.1038/s41584-020-0386-4