Studies on the pathophysiology of chronic D-penicillamine-induced myasthenia.
Studies on the pathophysiology of chronic D-penicillamine-induced myasthenia.
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慢性D-青霉胺诱发肌无力的病理生理学研究。
DOI：
10.1111/j.1749-6632.1981.tb33763.x
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发表时间：
1981
影响因子：
5.2
通讯作者：
Arnason,BG
中科院分区：
文献类型：
--
作者：
Burres,SA;Kanter,ME;Richman,DP;Arnason,BG
自1974年以来，人们认识到D-青霉胺（D-PA）治疗可能会并发重症肌无力样综合征。1970年以前，D-PA被用作治疗威尔逊病和胱氨酸尿症的螯合剂，这两种疾病并不常见。1970年后，它被广泛用于治疗类风湿性关节炎，可能会影响该疾病的免疫系统。在49例人D-PA诱导的肌无力（HPMG）病例中，45例发生在治疗类风湿性关节炎的患者中，3例发生在治疗威尔逊病的患者中，1例发生在治疗硬皮病的患者中（参见参考文献No.由于D-PA治疗引起的肌无力的报道越来越多，并且先前描述了这种药物改变免疫功能的能力，我们检查了它的一些特性，试图阐明HPMG的发病机制。以前，我们证明了D-PA治疗2至6个月的豚鼠对重复神经刺激的反应和微小终板电位（MEPP）振幅的降低。在目前的调查中，组织学和免疫学特征进行了检查，并试图将其与临床和电生理结果。
Since 1974, it has been recognized that D-penicillamine (D-PA) treatment might be complicated by a myasthenia gravis-like syndrome. Before 1970 D-PA was used as a chelating agent in the treatment of Wilson’s disease and cystinuria, two uncommon disorders. After 1970, it has been widely used as a treatment for rheumatoid arthritis, presumably affecting the immune system in that disease. Of the 49 cases of human D-PA-induced myasthenia (HPMG) 45 have occurred in patients treated for rheumatoid arthritis, three in patients treated for Wilson’s disease, and one in a patient treated for scleroderma (reviewed in References Nos. 1 and 2).Because of both the increasing number of reports of myasthenia resulting from D-PA therapy and the previously described ability of this drug to alter immune function, we examined some of its properties in an attempt to elucidate the pathogenesis of HPMG. Previously, we demonstrated both decremental responses to repetitive nerve stimulation and decreased miniature end-plate potential (MEPP) amplitudes in guinea pigs treated from two to six months with D-PA.~ In the present investigation, histologic and immunologic features were examined and an attempt made to correlate them with the clinical and electrophysiologic findings.