Incidence and natural history of Raynaud phenomenon: a long-term follow-up (14 years) of a random sample from the general population

Carpentier, Patrick; Satger, B.; Poensin, D; Maricq, Hildegard R.

doi:10.1016/j.jvs.2006.07.037

Cited by 65 publications

(25 citation statements)

References 21 publications

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“…Even in localized scleroderma, which has fewer systemic features, Raynaud’s progresses to peripheral ischemia with digital ulceration and loss of fingers [2]. Although the vasospasm associated with Raynaud’s phenomenon may be prodromal, it is not itself a sufficient cause of the disease because symptoms of vasospasm can precede diagnosis of limited scleroderma by many years, and the incidence of scleroderma in the population of people with Raynaud’s phenomenon is very low [3•]. …”

Section: Introductionmentioning

confidence: 99%

Is scleroderma a vasculopathy?

Fleming

Nash²,

Mahoney³

et al. 2009

Curr Rheumatol Rep

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Described as an autoimmune collagen vascular disease, the most striking feature of scleroderma may be a systemic vasculopathy. This vasculopathy includes characteristic noninflammatory macrovascular and microvascular changes with dramatic and possibly occlusive formation of a thickened neointima. Scleroderma vessels also have an unusual endothelial phenotype, with loss of normal markers including vascular endothelial (VE)-cadherin. These endothelial cells express type 1 interferon and regulator of G protein signaling 5 (RGS5), two molecules associated with vascular rarefaction. These genes may be important because tissue is hypoxic with high levels of vascular endothelial growth factor (VEGF), especially early in the disease. The combination of VEGF and rarefaction is not necessarily paradoxical. VEGF-mediated angiogenesis creates labile vessels that may not survive unless the vessel acquires a smooth muscle coat. The combination of interferon and RGS5 is consistent with an antiangiogenic phenotype. We offer a hypothesis that places vascular injury at the center of this disease and also suggest possible clinical approaches for arresting and/or reversing the disease.

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Section: Introductionmentioning

confidence: 99%

Is scleroderma a vasculopathy?

Fleming

Nash²,

Mahoney³

et al. 2009

Curr Rheumatol Rep

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“…The prevalence of Raynaud's phenomenon is said to be between 3% and 21% (Carpentier, 2006 Judy is a 39 year old social worker. After making the recommended lifestyle changes, she has not seen any improvement in her condition and is to be prescribed prophylactic immediate-release nifedipine.…”

Section: Questionmentioning

confidence: 99%

Raynaud's phenomenon

Nuttall¹

2017

Nurse Prescribing

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“…In longitudinal study from the authors, digital ulcers occurred in 5% of patients initially diagnosed with vasospastic RS when followed for more than 10 years, 41 with other epidemiologic studies demonstrating complete remission in one-third of patients, suggesting that vasospastic RS may be a transient phenomenon. 42 By contrast, patients with obstructive RS follow a more virulent course, with a 10-year incidence of digital ulcerations in half, and finger amputations in up to 25% of patients. Subsequent development of a connective tissue disease occurs in approximately one-third of patients who present with initial seropositivity.…”

Section: Expected Outcomesmentioning

confidence: 99%

Raynaud Syndrome

Valdovinos

Landry

2014

Techniques in Vascular and Interventional Radiology

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Raynaud syndrome (RS) was first described by the French physician Maurice Raynaud in 1862 with the characteristic tricolor change featuring pallor (ischemic phase), cyanosis (deoxygenation phase), and erythema (reperfusion phase) induced by cold or stress. Although the underlying pathophysiological mechanism is unclear, alterations in activity of the peripheral adrenoceptor have been implicated, specifically an enhanced smooth muscle contraction due to overexpression or hyperactivity of postsynaptic alpha 2 receptors. There are 2 ways that RS can appear clinically; isolated, formerly referred as Raynaud disease or now primary RS and in association with other conditions, usually connective tissue disorders (eg, Sjögren syndrome, systemic lupus erythematosus, scleroderma, and rheumatoid arthritis), frequently called Raynaud phenomenon or secondary RS. The estimated prevalence in the general population is 3%-5%, with a higher prevalence in women than in men. The diagnosis is mainly clinical, based on patient descriptions of skin changes. Upper extremity pulse-volume recording is used to rule out proximal arterial obstruction. The differentiation between a vasospastic vs and obstructive mechanism is made using digital pressures and photoplethysmography, where an obstructive mechanism has decreased pressures and blunted waveforms. Cold challenge testing, such as ice water immersion with temperature recovery, is highly sensitive but lack specificity. Serologic screening (antinuclear antibody and rheumatoid factor) is advocated to rule out associated connective tissue disorders. Most patients with RS can be managed conservatively, with avoidance of cold exposure or hand warming. For those in whom conservative management is inadequate, a number of pharmacologic and surgical therapies have been used. Owing to lack of complete understanding of the underlying pathophysiology, targeted therapy has not been possible; rather, therapy has been focused on the use of general vasodilation strategies. In this review, the diagnosis, natural history, and current medical and invasive therapy are summarized.

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Incidence and natural history of Raynaud phenomenon: a long-term follow-up (14 years) of a random sample from the general population

Abstract: These results show that, in the general population, RP is most often a benign condition and may disappear in a substantial proportion of subjects.

Cited by 65 publications

References 21 publications

Is scleroderma a vasculopathy?

Is scleroderma a vasculopathy?

Raynaud's phenomenon

Raynaud Syndrome

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