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The clinical relevance of Raynaud’s phenomenon symptom characteristics in systemic sclerosis

Abstract

Raynaud’s phenomenon (RP) is a cardinal feature of systemic sclerosis (SSc) and manifests with pain, digital colour change, sensory symptoms, and impaired function. SSc-RP is exacerbated by cold exposure (RP ‘attacks’) but many patients report persistent symptoms of background digital ischaemia. The aim of our study was to examine the significance of RP with digital colour change with or without symptoms, and persistent colour change in between attacks. Patients with SSc responses were obtained from the Patient Survey of experiences of Raynaud’s Phenomenon (PASRAP). We enquired about symptoms associated with Raynaud’s attacks, and persistent symptoms in between attacks. Data were analysed as descriptive statistics with appropriate parametric/non-parametric testing. Relevant PASRAP survey question data from 747 evaluable SSc patients from across three continents were analysed. Isolated colour change was rare (29/484, 6%). Digital ulcers were more common in SSc-RP associated with other sensory symptoms (42.1% vs. 24.1%, P=0.057) and more readily treated with phosphodiesterase-type 5 inhibitors (22.5% vs. 10.3%%, P=0.124). Over one-third of patients (n=92/239, 38%) reported persistent colour change in between Raynaud’s attacks. Patients with persistent colour change were more likely to have pulmonary arterial hypertension (15.2% vs. 7.5%, P=0.057) and be treated with calcium channel blockers (54.3% vs. 39.0%, P=0.021). SSc-RP with colour change and other symptoms and/or or persistent decolourisation in between attacks were more likely to have vascular complications of SSc and be treated with vascular therapies. Future research should explore the judicious use of vascular therapies as a potential form of disease modification in SSc.

Key Points
• Isolated colour change without other symptoms is rare in SSc patients.
• SSc patients often identify persistent symptoms in between attacks of RP.
• SSc-RP with colour change and other symptoms, or persistent decolourisation, may have greater disease severity and be treated with vascular therapies.

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Data Availability

The data underlying this article will be shared on reasonable request to the corresponding author.

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Acknowledgements

We thank the Scleroderma Foundation and Scleroderma and Raynaud’s UK for distributing the survey link to their members.

Funding

The study was funded by NIH/NIAMS K24 AR AR063120 to Dr. Dinesh Khanna.

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Correspondence to Michael Hughes.

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Ethics approval

The study was approved with exempt status by the University of Michigan IRB (Study ID: HUM00175143; OHRP IRB Registration Number(s): IRB00000246).

Conflict of interest

Michael Hughes—reports speaking fees from Actelion pharmaceuticals, Eli Lilly, and Pfizer, outside of the submitted work (all <$10,000). He is a member of a Data and Safety Monitoring Board for Certa Therapeutics. Suiyuan Huang—no reported conflicts of interest or competing interests. John Pauling—reports personal fees from Boehringer Ingelheim, Astra Zenaca, Sojournix Pharma and Permeatus Inc.; grants, personal fees, and non-financial support from Janssen; all outside the submitted work (<$10,000 for all honoraria / speaker fees). Maya Sabbagh—no reported conflicts of interest or competing interests. Dinesh Khanna—consulting fees from Acceleron, Actelion, Amgen, Bayer, Boehringer Ingelheim, Chemomab, CSL Behring, Genentech/Roche, Horizon, Paracrine Cell Therapy, Mitsubishi Tanabe Pharma, Prometheus, Theraly; Dr. Khanna is Chief Medical Officer of Eicos Sciences, Inc, a subsidiary of CiviBioPharma and has stock options.

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Hughes, M., Huang, S., Pauling, J.D. et al. The clinical relevance of Raynaud’s phenomenon symptom characteristics in systemic sclerosis. Clin Rheumatol (2022). https://doi.org/10.1007/s10067-022-06206-y

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  • DOI: https://doi.org/10.1007/s10067-022-06206-y

Keywords

  • Colour change
  • Management
  • Raynaud’s phenomenon
  • Symptoms
  • Systemic sclerosis
  • Treatment