[Anti-MDA5 (melanoma differentiation-associated gene 5) antibody and dermatomyositis with rapidly progressive interstitial pneumonia].
[Anti-MDA5 (melanoma differentiation-associated gene 5) antibody and dermatomyositis with rapidly progressive interstitial pneumonia].
复制标题
抗MDA5（黑色素瘤分化相关基因5）抗体与皮肌炎伴急进性间质性肺炎[J].
DOI：
10.2177/jsci.36.71
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
T. Mimori
中科院分区：
文献类型：
--
作者：
R. Nakashima;T. Mimori
抗MDA 5抗体是皮肌炎的特异性自身抗体之一，抗MDA 5阳性患者具有特征性的临床特征，如轻度肌炎、急性/亚急性间质性肺炎（A/SIP）发病率高、预后差、高铁蛋白血症和肝胆酶升高等。结果发现，抗-MDA 5阳性患者治疗前血清IL-6、IL-18、M-CSF、IL-10水平显著高于抗-MDA 5阴性患者，而血清IL-12、IL-22水平显著低于抗-MDA 5阴性患者。综合这些血清学结果，我们推测单核细胞和巨噬细胞活化可能是抗-MDA 5阳性患者病理生理学的基础。他们很少生存后，他们成为需要氧合，因此需要尽快治疗，一旦诊断已经作出。联合免疫抑制治疗（大剂量皮质类固醇、口服环孢素和静脉环磷酰胺（IVCY，900-1000 mg/m2，每隔一周））的强化方案提高了抗-MDA 5阳性患者的生存率。特别是，IVCY后约14天血清铁蛋白水平呈下降趋势，表明IVCY可能是治疗抗MDA 5阳性A/SIP患者的关键药物。
Anti-MDA5 antibody is one of the dermatomyositis-specific autoantibodies and anti-MDA5-potsitive patients show characteristic clinical features, such as hypomyositis, high prevalence of acute/subacute interstitial pneumonia (A/SIP) with poor prognosis, hyperferritinemia and elevated hepatobiliary enzyme. We found that serum IL-6, IL-18, M-CSF and IL-10 were significantly higher and serum IL-12 and IL-22 were significantly lower in anti-MDA5-positive patients than in anti-MDA5-negative patients before treatment. Taking together these serological findings, we hypothesized that monocyte and macrophage activation may underlie in the pathophysiology of anti-MDA5-positive patients. They rarely survive after they become to need oxygenation, and so need to be treated as soon as possible once the diagnosis has been made. Intensive regimen of combined immunosuppressive therapy (high-dose corticosteroids, oral cyclosporin and intravenous cyclophosphamide (IVCY, 900-1000 mg/m(2) in every other week)) improved the survival rate of anti-MDA5-positive patients. Especially, the serum ferritin levels tended to go down about 14 days after IVCY, suggesting that IVCY might be a key drug in the treatment of anti-MDA5-positive A/SIP patients.