Dermatomyositis and Polymyositis in Childhood

兒童期皮肌炎和多發性肌炎

鍾宏濤(Hung-Tao Chung) ； 黃璟隆(Jing-Long Huang) ； 王煇雄(Huei-Shyong Wang) ； 洪伯誠(Po-Cheng Hung) ； 周明亮(Min-Liang Chou)

《Acta Paediatrica Sinica》 35卷5期 (1994/10) Pp. 407-414

https://doi.org/10.7097/APS.199410.0407

摘要

兒童期皮肌炎(juvenile dermatomyositis)和多發性肌炎(polymyositis)為多系統發炎性疾病，以皮疹及近端肌肉無力為其臨床表現特徵，但其致病原因目前仍不清楚。近二十多年來因腎上腺皮質類醇廣泛使用，已使其預後有很大的改善。
台北長庚紀念醫院於民國70年(1981)至民國81年(1992)間，總共經歷了九例兒童期皮肌炎和多發性肌炎；其中七例皮肌炎，二例多發性肌炎。平均發病菌年齡為12歲（從7歲到16歲之間）。其中女童佔大多數，男童和女童的比例為3:6。臨應表現包括四肢肌肉無力、皮疹、眼部周圍水腫和吞嚥困難等。肌酸磷酵素(creatine phosphokinase, CPK)或乳酸脫氫酵素(lactic dehydrogenase, LDH)均有顯昇高的現象。有七例曾接受肌電圖和肌肉切片檢查，其結果均符合皮肌炎和多發性肌炎的診斷特徵。
九位患童在診斷确定後，均接受腎上腺皮質類醇(prednisolone)的治療。治療時間從三個月到六十五個月不等（平均治療時間為25.3個月）。其中二例除腎上腺皮質類醇外，亦接受免疫抑制劑hydroxychloroquine和azathioprine的治療。上前有六例沒有接受任何治療且無任何臨床癓兆；二例仍在接受腎上腺皮質類醇及免疫抑制的治療；一例在診斷六個月後因原發性腹膜炎而死亡。
結論是，兒童期皮肌炎和多發性肌炎較好發於女童；而以單一週期的臨床病程最常見。整體而言，其預後是相當不錯的。

關鍵字

皮肌炎 ； 多發性肌炎

並列摘要

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years(range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.

並列關鍵字

dermatomyositis ； polymyositis