Leys D, Hatron P Y, Parent M, Pasquier F, Krivosic I, Petit H
Rev Neurol (Paris). 1984;140(11):670-2.
A 61 year old patient presented with muscular atrophy and weakness, predominant at the shoulder girdle, as the first manifestation of a progressive systemic sclerosis with the signs of the CREST syndrome: subcutaneous calcinosis, Raynaud phenomenon, esophagus disorders, sclerodactyly, telangiectasia. The histological features were consistent with polymyositis. In spite of corticotherapy, total recovery was not obtained. The nosological relationships with Mixed Connective Tissue Disease is discussed. Myositis is infrequent in progressive systemic sclerosis and corticosteroid therapy is discussed.
一名61岁患者出现肌肉萎缩和无力,以肩胛带为主,这是进行性系统性硬化症伴CREST综合征体征(皮下钙质沉着、雷诺现象、食管疾病、指(趾)硬皮病、毛细血管扩张)的首发表现。组织学特征符合多发性肌炎。尽管进行了皮质激素治疗,但未完全恢复。文中讨论了与混合性结缔组织病的病种关系。进行性系统性硬化症中肌炎并不常见,并对皮质类固醇治疗进行了讨论。