Corbo M, Nemni R, Iannaccone S, Quattrini A, Lodi M, Praderio L, Comola M, Lorenzetti I, Comi G, Canal N
Department of Neurology, State University of Milan, Istituto Scientifico H. San Raffaele, Italy.
Clin Neuropathol. 1993 Mar-Apr;12(2):63-7.
Nervous system involvement is rare in progressive systemic sclerosis (PSS). We present a clinical pathological and immunological study of two patients with peripheral sensory motor neuropathy and PSS. In both, the sural nerve biopsies showed axonal degeneration with increased endoneurial connective tissue. There were also clusters of myelinated fibres indicating axonal regeneration. Only mild microangiopathic changes were evident in the endo, peri and epineurial vessels. By Western immunoblots, patients' sera contained a band of reactivity to a protein from peripheral nerve identified as collagen type I. Primary involvement of the peripheral nerves during PSS is very unusual. Abnormal production of collagen tissue and presence of microvascular disease are considered to be two possible causes of neuropathy. We think that our results suggest the important role of the connective tissue proliferation in the pathogenesis of PSS neuropathy.
神经系统受累在进行性系统性硬化症（PSS）中较为罕见。我们对两名患有周围感觉运动神经病变和PSS的患者进行了临床病理和免疫学研究。在这两名患者中，腓肠神经活检均显示轴突变性伴神经内膜结缔组织增多。也有成群的有髓纤维，提示轴突再生。仅在内膜、束膜和外膜血管中可见轻度微血管病变。通过蛋白质免疫印迹法，患者血清中出现一条与周围神经中一种被鉴定为I型胶原的蛋白质发生反应的条带。PSS期间周围神经的原发性受累非常罕见。胶原组织的异常产生和微血管疾病的存在被认为是神经病变的两个可能原因。我们认为我们的结果提示结缔组织增殖在PSS神经病变发病机制中起重要作用。
