进行性系统性硬化症(PSS)是一种系统性结缔组织病,主要累及皮肤、胃肠道、肺和肾脏。与其它胶原性疾病不同,其神经系统表现不常见,发生率仅0.8%至5.6%,且主要为肌肉改变和外周神经损害,中枢神经系统受累罕见,仅有几例大脑动脉炎的报道。本文报道PSS 的神经系统表现发生率较高,讨论了这种系统性疾病和其神经系统并发症间的可能关系。 Progressive systemic sclerosis (PSS) is a systemic connective tissue disease involving the skin, the gastrointestinal tract, the lungs, and the kidneys. Unlike other collagen diseases, neurological manifestations are uncommon, with rates of only 0.8% to 5.6% and are mainly muscle changes and peripheral nerve damage. Central nervous system involvement is rare and only a few cases of cerebral arteritis are reported. This article reports the higher incidence of neurological manifestations of PSS and discusses the possible relationship between this systemic disease and its neurological complications.