How common is Raynoud's syndrome?

1.

Elsevier ClinicalKey Derived Clinical Overview

• RP is classified clinically into primary or secondary forms and affects approximately 3% to 5% of the general population, 15% of children younger than 12 yr, and less than 1% of adults older than 60 yr. • Occurs more commonly in colder climates. • Primary RP usually occurs between the ages of 12 and 25 yr. • It is more likely to affect more women than men (4:1). • 5% to 15% of patients with primary RP develop a secondary cause later in the course of the disease (mostly a connective tissue disorder). • Secondary RP tends to begin after age 35 to 40 yr. • Secondary RP occurs in more than 90% of patients with scleroderma and in approximately 30% of patients with systemic lupus erythematosus or Sjögren syndrome.

2.

Bartholomew, John R. (2024). In Goldman-Cecil Medicine (pp. 467). DOI: 10.1016/B978-0-323-93038-3.00066-6

Raynaud phenomenon is defined as episodic attacks of discoloration (white or blue) of the digits brought on by cold or emotional stimuli and resulting in a characteristic triphasic color change from white to blue to red. Raynaud phenomenon affects 3 to 5% of the U.S. population and is seen more often in young women, in whom it is reported to have a prevalence as high as 5 to 15%. The prevalence is higher in cooler northern climates and in smokers. Family history (as many as 30 to 50% of patients with primary Raynaud have a first-degree relative with this condition), estrogen exposure, and emotional stress are commonly associated with Raynaud phenomenon in women. The pathophysiology of the vasoconstriction in Raynaud phenomenon is not well understood, but variants in genes related to α2A-adrenoceptor signalling may play a role.It includes abnormalities of the blood vessel wall, neural control mechanisms, and intravascular factors, including platelet activation and oxidative stress. As the arterial vasoconstriction subsides, postcapillary venular constriction leads to deoxygenation of the blood and the cyanotic appearance. On rewarming, blood flow increases as a result of vasodilation, which leads to the red or hyperemic appearance of the digits. Primary Raynaud phenomenon is a benign vasospastic disorder, whereas a number of conditions are associated with the secondary form (Table 66-4). Several clinical features can help distinguish between these two forms (Table 66-5).

3.

Curtiss P, Svigos K, Schwager Z, Lo Sicco K, Franks AG. Journal of the American Academy of Dermatology. 2024;90(2):223-234. doi:10.1016/j.jaad.2022.06.1199.

Publish date: February 4, 2024.

Raynaud's phenomenon (RP) is a relatively common disease with both primary and secondary forms. It is well understood as a vasospastic condition affecting the acral and digital arteries, resulting in characteristic, well-demarcated color changes typically in the hands and feet in response to cold or stress. Secondary RP (SRP) has been described in association with a variety of rheumatologic and nonrheumatologic diseases, environmental exposures, and/or medications. While both primary RP and SRP may impact the quality of life, SRP may lead to permanent and potentially devastating tissue destruction when undiagnosed and untreated. It is therefore crucial for dermatologists to distinguish between primary and secondary disease forms early in clinical evaluation, investigate potential underlying causes, and risk stratify SRP patients for the development of associated autoimmune connective tissue disease. The epidemiology, pathogenesis, and clinical presentation and diagnosis of both forms of RP are described in detail in this review article.

4.

St. Clair, E. William (2024). In Goldman-Cecil Medicine (pp. 1783). DOI: 10.1016/B978-0-323-93038-3.00247-1

Extraglandular manifestations of primary Sjögren syndrome occur in approximately 50% of patients with this disease, although only about 15% develop serious organ system involvement. Fatigue is the most common extraglandular symptom. Raynaud syndrome (Chapter 66) occurs in up to one-third of patients and may precede the onset of sicca symptoms by several years. Polyarthralgia/polyarthritis occurs commonly in Sjögren syndrome and may precede its diagnosis. The joints most frequently affected are the wrists, hands, ankles, and feet. The degree of synovial inflammation is generally much less severe than in rheumatoid arthritis and does not lead to erosive joint damage. The types of skin rash are varied and include annular erythema, subacute cutaneous lupus erythematosus, and cutaneous vasculitis. Annular erythema appears in different forms: a donut-ring erythema with an elevated border, a polycyclic-like erythema with marginal scaling, and a papular lesion resembling an insect bite. These lesions are not photosensitive and resolve without residual hyperpigmentation. Subacute cutaneous lupus erythematosus is associated with photosensitivity, the presence of anti-Ro/SSA antibodies, and papulosquamous lesions with scaling or with annular, polycyclic lesions with marginal scale and central clearing. Cutaneous vasculitis may present as palpable purpura, ulcers, erythematous papules and macules, and urticarial vasculitis. Urticarial vasculitis may be distinguished from allergic urticaria by the persistence of these lesions at a single location for more than 24hours and by their tendency to leave a bruise. Nonpalpable purpura may occur in patients with high levels of serum immunoglobulins. Lung involvement in primary Sjögren syndrome most frequently takes the form of small airways disease or interstitial lung disease (Chapter 80),especially nonspecific interstitial pneumonitis and usual interstitial pneumonitis.

5.

Garner R, Kumari R, Lanyon P, Doherty M, Zhang W. BMJ Open. 2015;5(3):e006389. doi:10.1136/bmjopen-2014-006389.

Publish date: March 1, 2015.

OBJECTIVE: To systematically review the literature with regard to the prevalence, incidence, risk factors and associations of primary Raynaud's phenomenon (PRP). METHOD: A systematic review of the literature of observational studies for PRP was undertaken using five electronic databases. Any studies reporting prevalence, incidence and risk factors of PRP were collected. Relative risk or OR and 95% CI were extracted or calculated to present the association between risk factors and PRP. Random eff... (truncated preview)