Raynaud Syndrome

Abstract

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.

Cold sensitivity of TRPA1 is unveiled by the prolyl hydroxylation blockade-induced sensitization to ROS

“…Since DMOG, which does not induce ROS generation, is sufficient to induce ROS- and TRPA1-dependent cold hypersensitivity in mice, oxalate, a characteristic metabolite of L-OHP, seems responsible for the acute cold hypersensitivity 24 45 46 47 48 via PHD inhibition. The molecular mechanism underlying hypoxia- and PHD-mediated sensitization of sensory TRPA1 to cold and ROS may also underlie the pathophysiological process of cold hypersensitivity induced by peripheral ischaemia, such as in Buerger's disease 49 and Raynaud syndrome 50 .…”

Postocclusive Hyperemia Measured with Laser Doppler Flowmetry and Transcutaneous Oxygen Tension in the Diagnosis of Primary Raynaud’s Phenomenon: A Prospective, Controlled Study

“…However, evidence suggests that the mechanism in primary RP may involve an overexpression or hyperactivity of postsynaptic alpha-2 adrenergic receptors in the smooth muscle of digital vessels [4, 5]. …”

Assessment of quality of life in patients with Raynaud’s phenomenon after procedures with physical therapy

“…6 U osób długo zmagających się z objawem Raynauda, niepoddających się odpowiedniemu leczeniu lub u których leczenie nie przynosi zamierzonych rezultatów, pojawiają się zmiany martwicze, zgorzele i trudno gojące się rany. 8 W typowym przebiegu choroby wyróżnia się 3 etapy: zblednięcia, zasinienia oraz czynnego przekrwienia. Pierwszym etapem jest napadowe zblednięcie palców, czyli zmniejszenie przepływu krwi, co prowadzi do osła-bienia czucia powierzchownego i uczucia dyskomfortu u pacjenta.…”