幼年型皮肌炎及多發性肌炎之臨床觀察

Clinical Observation of Juvenile Dermatomyositis and Polymyositis

本報告收集自1970年至1987年間在臺大小兒科診治之幼年型皮肌炎及多發性肌炎共十一例。分析其臨床症狀，生化學免疫學及病理學之檢查結果，並參考文獻做一回顧。結果如下：1)11例中男性3例，女性8例，發病年齡從3歲到16歲，平均8歲；2)臨床症狀以肌無力(100％)， 皮疹(82％)，肌疼痛(64％)，發燒(55％)及吞嚥或發音困難(36％)最為常見；3)由多項線性廻歸統計分析中顯示病人初次住院時之肌相關酵素值（CPK, LDH, SGOT等）之高低和以後肌功能之是否能完全恢復沒有明顯相關，又初期CPK，LDH值雖與肌病變中細胞侵蝕之 程度呈有意義之相關，但肌肉病變的輕重卻與初期肌功能障礙程度卻呈沒有意 義之相關性；4)本病患者若併有皮膚血管炎時，則此種病變很難痊癒；5)本病 之病理切片檢查，發現除肌纖維之變化外，常併有種種血管病變，顯示血管病 變在幼年型皮肌炎及多發性肌炎之致病機轉上扮演某種重要角色；6)與成年型 比較本病患者很少合併有其他自體免疫疾病或惡性腫瘤，但27％併有皮膚鈣化 現象，較成年多見；7)3例曾接受免疫學檢查，有2例在急性期T細胞數目有減少現象，治療後逐漸恢復；8)有1例CPK持續增高已超過1年，雖然肌功能在治療半年後已恢復正常。但這類病患具有高潛在性復發之可能，故應定期追蹤其相關酵素；9)幼年型皮肌炎及多發性肌炎預後比成年型好，在本科追蹤5個月以上，最長10年的8例中，肌功能幾乎全部恢復，沒有死亡病例。

From 1970~1987, 11 cases of juvenile dermatomyositis (JDMS) and polymyositis (PM) were diagnosed at The National Taiwan University Hospital and followed up for 2 wks to 10 yrs. The clinical manifestations, biochemical and immunological data were aralysed. There was no correlation between initial muscle enzyme levels and the final functional disability. Cutaneous vasculitis occurred in 2 cases and the skin lesions were difficult to resolve. A 10 years old girl who had initial high ANF (1:640) and positive RA factor developed JRA 4 years after complete remission of JDMS. Patholoy of muscle biopsy ranged from minimal or tiny focal cellular infiltration to massive muscle necrosis with cell infiltration, vascalar occlusion and thrombosis. Furthermore, there was no correlation between pathological changes and initial muscle enzyme levels.