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Intravenous Immunoglobulin Therapy in Juvenile Dermatomyositis

以靜脈注射免疫球蛋白治療幼年型皮肌炎

摘要


爲了解靜脈注射免疫球蛋白在幼年型皮肌炎的療效,有七個病人接受這項治療。他們的適應症包括:病情惡化、無法降低類固醇的劑量、取代其它免疫抑制劑,及早期用在病情嚴重的病人。兩個病人有緩慢的改善,他們在類固醇的劑量大量降低之後仍保持穩定。一個病人只有在前三次有尚可的反應;有三個病人有明顯、快速的反應;另一個病人則沒有進步。不過,有四個本來有改善的病人,在靜脈注射免疫球蛋白的治療結束後,發生病情惡化的現象,不良反應包括發燒(兩次),及暫時性的蛋白尿(兩次)。總而言之,就它快速的效果、具有降低類固醇或其它免疫抑帛劑的潛力而言,以靜脈注射免疫球蛋白治療幼年型皮肌炎可能有嘗試的價值。不過就本觀察看來,這治療似乎不能改變幼年型皮肌炎的自然病程。

並列摘要


To investigate the effects of intravenous immunoglobulin (IVIG) on patients of juvenile dermatomyositis (JDM), seven children with JDM were given monthly IVIG in conjunction with other treatments. The indications included disease exacerbation, inability to reduce dose of steroid, replacing cytotoxic drug because of complication and remission-induction for those with severe initial manifestations. Two patients were reported to improve slowly and maintained improved status while on a markedly reduced dose of steroid. One patient responded favorably to the first three courses only. Three patients had evident and quick responses, which could be seen as early as two or three days after the infusion. One patient failed to experience any beneficial effect by IVIG. Four initial responders had an aggravation some time after discontinuing the monthly regimen. The untoward reactions included only two occasions of fever, and another two occasions of fever associated with transient proteinuria. In conclusion, IVIG may be of value in terms of its quick, pulsatile effect and the adjuvant potential to reduce the side effect resulting from exposure to steroid or other immunosuppressive agents as well. However, its long-term efficacy is doubtful, based on this observation.

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