Goodfield M J, Orchard M A, Rowell N R
Department of Dermatology, General Infirmary, Leeds.
Br J Haematol. 1993 Aug;84(4):675-80. doi: 10.1111/j.1365-2141.1993.tb03145.x.
It is unclear whether the changes in platelet function which are observed in systemic sclerosis are a primary characteristic of this disease or whether they occur secondary to vascular changes. Whole blood platelet aggregation was studied in 26 patients with systemic sclerosis, normal subjects matched for age, sex and secondary characteristics, 19 patients with Raynaud's disease and 19 patients with systemic lupus erythematosus. Plasma levels of fibrinogen, von Willebrand factor antigen and factor VIII:C were also measured. Systemic sclerosis was associated with a significant (P > 0.001) enhancement of the sensitivity of platelets to collagen. In contrast, significant enhancement of the response to either ADP or adrenaline was not observed. Enhanced sensitivity to collagen was not associated with the presence of either Raynaud's disease or systemic lupus erythematosus. Systemic sclerosis was associated with significantly raised levels of von Willebrand factor antigen and fibrinogen. On an individual patient basis, von Willebrand factor antigen was related to the severity of the disease whereas platelet sensitivity to collagen was not. In conclusion, this study suggests that the enhanced sensitivity to collagen which occurs in systemic sclerosis is due to a primary change in the platelet and that this change can combine with elevated levels of adhesive proteins.
目前尚不清楚系统性硬化症中观察到的血小板功能变化是该疾病的主要特征，还是继发于血管变化。对26例系统性硬化症患者、年龄、性别和次要特征相匹配的正常受试者、19例雷诺病患者和19例系统性红斑狼疮患者进行了全血血小板聚集研究。还测量了血浆纤维蛋白原、血管性血友病因子抗原和因子VIII:C水平。系统性硬化症与血小板对胶原蛋白敏感性的显著增强（P>0.001）相关。相比之下，未观察到对ADP或肾上腺素反应的显著增强。对胶原蛋白的敏感性增强与雷诺病或系统性红斑狼疮的存在无关。系统性硬化症与血管性血友病因子抗原和纤维蛋白原水平的显著升高相关。在个体患者中，血管性血友病因子抗原与疾病严重程度相关，而血小板对胶原蛋白的敏感性则不然。总之，本研究表明，系统性硬化症中出现的对胶原蛋白敏感性增强是由于血小板的原发性变化，并且这种变化可与粘附蛋白水平升高相结合。
