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Epidemiology of Raynaud’s Phenomenon

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Raynaud’s Phenomenon

Abstract

The prevalence of Raynaud’s phenomenon (RP) in most studies of the general population is between 3 and 5 %. Primary RP is reversible vasospasm in peripheral arteries occurring in the absence of an underlying disease and accounts for 80–90 % of cases. Secondary RP develops in association with an underlying disorder and is often characterised by structural vascular abnormalities and irreversible vascular occlusion. The prevalence of primary RP ranges from 2 to 20 % in women and 1–12 % in men depending on geographic location, the population studied, the definition of RP used and the method of case ascertainment.

In women, the onset of RP is more commonly at an early age and is associated with a family history of RP suggesting genetic factors may play a role, as may hormonal and emotional factors. RP secondary to autoimmune disease is also more common in women than in men. In contrast, the prevalence of RP in men increases with increasing age and smoking and is more likely to be secondary to occupational exposures such as vibration or atherosclerotic peripheral vascular disease than in women. Low body weight is a risk factor in both sexes. Studies of risk factors have been hindered by poor methodology such as cross-sectional study design.

The prevalence of secondary RP is related to the underlying disease. Progression to secondary RP occurs in 14–37 % of subjects with primary RP. Almost 99 % of patients who progress develop an autoimmune disease, most commonly systemic sclerosis (SSc). Risk factors for progression include positive ANA, elevated ESR, SSc-specific autoantibodies and abnormal nailfold capillaroscopy.

Primary RP follows a relatively benign course with minimal impact on function and quality of life. The greatest impact of secondary RP on morbidity and function arises from complications from digital ulceration and ischemic necrosis, namely pain, infection, gangrene and amputation, with resultant loss of hand function.

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Abbreviations

ANA:

Anti-nuclear antibody

CI:

Confidence interval

CTD:

Connective tissue disease

DU:

Digital ulceration

ESR:

Erythrocyte sedimentation rate

GP:

General practice

HAQ:

Health assessment questionnaire

MCTD:

Mixed connective tissue disease

ND:

Not described

OR:

Odds ratio

RA:

Rheumatoid arthritis

RP:

Raynaud’s phenomenon

RR:

Relative risk

SLE:

Systemic lupus erythematosus

SSc:

Systemic sclerosis

UK:

United Kingdom

US:

United States of America

VCM:

Vinyl chloride monomer

VWF:

Vibration white finger

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Authors and Affiliations

  1. Department of Rheumatology, Royal Adelaide Hospital, North Terrace, Adelaide, SA, 5000, Australia

    Adam Maundrell & Susanna M. Proudman

  2. Discipline of Medicine, University of Adelaide, Adelaide, SA, 5000, Australia

    Susanna M. Proudman

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  1. Adam Maundrell
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  2. Susanna M. Proudman
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Correspondence to Susanna M. Proudman .

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Editors and Affiliations

  1. School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  2. Department of Rheumatology, The University of Manchester, Manchester, United Kingdom

    Ariane L. Herrick

  3. Department of Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medic, Baltimore, Maryland, USA

    Nicholas A. Flavahan

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Maundrell, A., Proudman, S.M. (2015). Epidemiology of Raynaud’s Phenomenon. In: Wigley, F., Herrick, A., Flavahan, N. (eds) Raynaud’s Phenomenon. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1526-2_3

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  • DOI: https://doi.org/10.1007/978-1-4939-1526-2_3

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