Pathogenesis of Raynaud's phenomenon

Abstract

The pathogenesis of Raynaud's phenomenon is not fully understood. However, the last 20 yr have witnessed enormous increases in our understanding of different mechanisms which, singly or in combination, may contribute. A key point is that Raynaud's phenomenon can be either primary (idiopathic) or secondary to a number of underlying conditions, and that the pathogenesis and pathophysiology vary between these conditions. This review concentrates upon those subtypes of Raynaud's phenomenon of most interest to rheumatologists: systemic sclerosis-related Raynaud's phenomenon, primary Raynaud's phenomenon and Raynaud's phenomenon secondary to hand-arm vibration syndrome. In this review, I shall discuss the main mechanisms thought to be important in pathophysiology under the three broad headings of 'vascular', 'neural' and 'intravascular'. While these are false distinctions because all interrelate, they facilitate discussion of the key elements: the blood vessel wall (particularly the endothelium), the neural control of vascular tone, and the many circulating factors which can impair blood flow and/or cause endothelial injury. Vascular abnormalities include those of both structure and function. Neural abnormalities include deficiency of the vasodilator calcitonin gene-related peptide (released from sensory afferents), alpha(2)-adrenoreceptor activation (possibly with up-regulation of the normally 'silent' alpha(2C)-adrenoreceptor) and a central nervous system component. Intravascular abnormalities include platelet activation, impaired fibrinolysis, increased viscosity and probably oxidant stress. As our understanding of the pathophysiology of Raynaud's phenomenon increases, so do our possibilities for identifying effective treatments.

Radiofrequency upper thoracic sympathectomy in the treatment of critical upper limb ischemia - a case series

“…In general, genetic factors, hormonal factors (mainly women are affected), mental stress and smoking contribute to the development of the disease. Mechanisms and causes of Raynaud's phenomenon may be divided to vascular, neurogenic and intravascular 9 . Vascular causes included changes in the structure of the vascular wall as well as the function of endothelium accompanied by a local deficit of prostacycline and nitrogen oxide 10 or increased level of angiotensin II (ref.…”

Raynaud’s syndrome, 2011

“…Az endothelium, a fi zikai barrier szerepén túlmenően, számos fontos kémiai anyag termelése és felszabadítása által kiemelkedő funkciót tölt be a keringés szabályozásában. Az endothelfüggő folyamatok egyensúlyának megbomlása, a vasoconstrictor hatású mediátorok (például endothelin-1, tromboxán-A2) túltermelődése, illetve a vasodilatator hatású faktorok (NO, prosztaciklin) csökkent jelenléte kulcsszerepet játszhat a Raynaud-jelenség kifejlődésében [10]. A primer formára jellemző tisztán funkcionális eltérésekkel szemben a szekunder, autoimmun betegségekhez társult formák esetében a kórfolyamat előreha-ladtával az endothel által fokozottan termelt vasoconstrictor faktorok, illetve az alapbetegség hatására megváltozott citokinmiliő (például emelkedett tumor growth factor-beta [TGF-béta] szintek) az ujjak érfalá-nak strukturális károsodásához, megvastagodásához, illetve lumenének elzáródásához vezetnek.…”

Fenómeno de Raynaud

“…El fenómeno de Raynaud se caracteriza por una disminución de la circulación sanguínea en los dedos de las manos y de los pies, produciéndose dolor y cambios de coloración que en ocasiones pueden afectar a otras zonas como labios, nariz, lóbulos de las orejas, lengua y mentón [1][2][3][4][5] .…”