Prevalence, Incidence, and Mortality of Raynaud’s phenomenon, Sjögren’s syndrome, and scleroderma: an umbrella review of systematic reviews

Chen, Anthony; Lax, Stephanie J; Grainge, Matthew J; Lanyon, Peter C; Pearce, Fiona A

Abstract

Objectives:

To comprehensively review systematic reviews of prevalence, incidence, and mortality of Raynaud’s, Sjögren’s, and Scleroderma, and to identify any research gaps.

Methods:

An umbrella review of English language systematic reviews was undertaken using PubMed and Embase (OVID) covering the period 2000-2023 (PROSPERO CRD42023434865). The estimate and its corresponding 95% confidence interval were reported when available from each systematic review. The quality of systematic reviews was assessed using the Scottish Intercollegiate Guidelines Network (SIGN) tool. A narrative synthesis was undertaken.

Results:

17 systematic reviews were identified, of which one was for Raynaud’s, five for Sjögren’s and 11 for Scleroderma. There were some high-quality systematic reviews for Sjögren’s and mortality of Scleroderma. However, there were only low-quality systematic
reviews of prevalence and incidence of Raynaud’s and Scleroderma. Furthermore, there were no systematic reviews for the mortality of Raynaud’s. For Raynaud’s, the pooled prevalence was 4850 per 100,000; pooled annual incidence was 250 per 100,000. For Sjögren’s, prevalence was 60-70 per 100,000; annual incidence was 6.92 per 100,000 and the pooled standardised mortality ratio ranged from 1.38-1.48. For Scleroderma, pooled prevalence ranged from 17.6-23 per 100,000; annual incidence was 1.4 per 100,000; and the pooled standardised mortality ratio ranged from 2.72-3.53.

Conclusion:

The outcomes of Raynaud’s were less-well described compared to Sjögren’s and Scleroderma. There was a lack of high-quality systematic reviews for the prevalence and incidence of Raynaud’s and Scleroderma. Therefore, further studies and systematic reviews with rigorous case definitions, assessing different ethnic groups are warranted in this area.

Citation

Chen, A., Lax, S. J., Grainge, M. J., Lanyon, P. C., & Pearce, F. A. (2024). Prevalence, Incidence, and Mortality of Raynaud’s phenomenon, Sjögren’s syndrome, and scleroderma: an umbrella review of systematic reviews. Rheumatology Advances in Practice, 8(3), Article rkae086. https://doi.org/10.1093/rap/rkae086

• Journal Article Type: Article
• Acceptance Date: Jul 1, 2024
• Online Publication Date: Jul 27, 2024
• Publication Date: Jul 27, 2024
• Deposit Date: Jul 9, 2024
• Publicly Available Date: Jul 10, 2024
• Journal: Rheumatology Advances in Practice
• Electronic ISSN: 2514-1775
• Publisher: Oxford University Press
• Peer Reviewed: Peer Reviewed
• Volume: 8
• Issue: 3
• Article Number: rkae086
• DOI: https://doi.org/10.1093/rap/rkae086
• Keywords: Umbrella review, Prevalence, Incidence, Mortality, Raynaud’s, Sjögren’s, and Scleroderma
• Public URL: https://nottingham-repository.worktribe.com/output/37146850