Generini S, Kahaleh B, Matucci-Cerinic M, Pignone A, Lombardi A, Ohtsuka T
Istituto di Clinica Medica IV, Università degli Studi di Firenze.
Ann Ital Med Int. 1996 Apr-Jun;11(2):125-31.
In systemic sclerosis, the exaggerated generalized vasospastic tendency is clinically represented by Raynaud's phenomenon as shown by an early digital arterial closure after cold stimulation, and by an inadequate vasodilatory response to heat. The phenomenon is not restricted to the extremities, and can also occur in internal organs. Repeated attacks of Raynaud's phenomenon may contribute to vascular disease in systemic sclerosis by a mechanism of reperfusion injury of the endothelium, and may contribute to tissue fibrosis. Although the aetiology and pathogenesis of Raynaud's phenomenon remain unknown, recent advances in the understanding of mechanisms of vascular tone control provide us with an opportunity to reconsider the pathogenetic process of Raynaud's phenomenon. It is now clear that neuropeptides, the vascular endothelium, and platelets are the three major contributors to the control of vascular tone. Our hypothesis suggests the presence of a sensory nervous system failure, leading to an unopposed endothelial and platelet control of vascular tone. Endothelial injury and platelet activation in systemic sclerosis lead to a shift in vascular function to a pro-vasospastic function not balanced by a vasodilatory sensory input; thus, enhanced vasospasm is generated. The investigation of the role of local vascular mediators in vasospasm may lead to a better understanding of vascular tone control and of Raynaud's phenomenon pathophysiology in systemic sclerosis.
在系统性硬化症中，过度的全身性血管痉挛倾向在临床上表现为雷诺现象，如冷刺激后早期手指动脉闭塞，以及对热的血管舒张反应不足。该现象不仅限于四肢，也可发生在内脏器官。雷诺现象的反复发作可能通过内皮细胞再灌注损伤机制导致系统性硬化症中的血管疾病，并可能导致组织纤维化。尽管雷诺现象的病因和发病机制尚不清楚，但对血管张力控制机制的最新认识进展为我们重新审视雷诺现象的发病过程提供了机会。现在很清楚，神经肽、血管内皮和血小板是控制血管张力的三大主要因素。我们的假设表明存在感觉神经系统功能障碍，导致血管张力不受内皮和血小板控制的影响。系统性硬化症中的内皮损伤和血小板活化导致血管功能向血管痉挛功能转变，而血管舒张感觉输入无法平衡这种转变；因此，产生了增强的血管痉挛。研究局部血管介质在血管痉挛中的作用可能有助于更好地理解血管张力控制以及系统性硬化症中雷诺现象的病理生理学。
