Treatment of Juvenile Dermatomyositis: Neurologist's View Point

幼年性皮肌炎的治療－神經科的觀點

簡穎瑄(Yin-Hsuan Chien) ； 曾慶昌(Ching-Chang Tseng)

《北市醫學雜誌》 6卷5期 (2009/10) Pp. 398-408

摘要

幼年性皮肌炎是發炎性肌肉病變的一種，主要影響皮膚、肌肉，和其他組織像是食道、肺部等等。因為幼年性皮肌炎的病人的數目不多，所以很難有一個治療的規範。此篇文章主要在討論目前對於這個疾病的新知，臨床表現，診斷，治療，尤其是以神經科醫師的角度來看。初步治療一般先用口服的類固醇，但是劑量、還有調整劑量的速度都尚未有一個定論。其他的免疫抑制劑或是免疫調節劑，尤其是包括免疫球蛋白等等藥物，常用在復發性的肌肉炎的病人。另外還有一些新的免疫抑制劑正在發展當中，對於治療成效還未有結論。皮肌炎的治療需要免疫學者及神經科醫師等專家來共同評估，治療的原則還需要實症醫學主導的研究及長期追蹤的報告。

關鍵字

幼年性皮肌炎 ； 免疫抑制劑 ； 皮質類固醇

並列摘要

Juvenile dermatomyositis is an inflammatory myopathy that mainly affects skin and muscle, but may also affect other tissues. This review describes current knowledge of the clinical presentation, diagnosis and treatment of dermatomyositis, especially from a neurological aspect of view. Oral corticosteroid is the mainstay of initial treatment. However, the dose, tapering rate and treatment duration remains under debate. Other immunosuppressant and immunomodulatory agents include azathioprine, methotrexate, cyclosporine, intravenous immunoglobulin and plasma exchange are also used to treat refractory myositis. Newer immunosuppressive agents such as tumor necrosis factor a blockade are promising. The treatment goals need to be set by specialists including immunologists and neurologists. Evidence-based and long-term follow-up data would be helpful to making clinical treatment choices in the future.

並列關鍵字

Juvenile Dermatomyositis ； immunosuppressant ； corticosteroid