Kikuchi Shiori, Sawada Jun, Saito Tsukasa, Katayama Takayuki, Fujishiro Daisuke, Nishino Ichizo, Hasebe Naoyuki
Internal Medicine, Cardiovascular, Respiratory and Neurology Division, Asahikawa Medical University.
Division of Metabolism and Biosystemic Science, Department of Medicine, Asahikawa Medical University.
Rinsho Shinkeigaku. 2021 Apr 21;61(4):228-233. doi: 10.5692/clinicalneurol.cn-001509. Epub 2021 Mar 25.
A 62-year-old Japanese man with swollen fingers and walking difficulty due to myalgia and muscle weakness in proximal limb muscles was admitted to our hospital. Serum creatine kinase was remarkably increased (7,380 U/l) and rapidly progressing interstitial pneumonia developed. Muscle biopsy showed necrotic and regenerating fibers without mononuclear infiltration and fibrosis. Anti-Th/To antibodies were detected in the serum, and anti-Th/To antibody-positive systemic sclerosis was diagnosed. Anti-Th/To antibody-positive sclerosis-associated myopathy has not yet been reported in the literature. The present case suggests that anti-Th/To antibody-positive systemic sclerosis can be accompanied by immune-mediated necrotizing myopathy and be effectively treated with immunotherapy comprising corticosteroids, tacrolimus and immunoglobulin.
一名62岁的日本男性因近端肢体肌肉疼痛和肌无力导致手指肿胀及行走困难，入住我院。血清肌酸激酶显著升高（7380 U/l），并出现快速进展的间质性肺炎。肌肉活检显示有坏死和再生纤维，无单核细胞浸润和纤维化。血清中检测到抗Th/To抗体，诊断为抗Th/To抗体阳性的系统性硬化症。抗Th/To抗体阳性的硬化症相关肌病在文献中尚未见报道。本病例提示，抗Th/To抗体阳性的系统性硬化症可伴有免疫介导的坏死性肌病，采用包括皮质类固醇、他克莫司和免疫球蛋白的免疫疗法可有效治疗。
