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Tacrolimus rescue therapy for severe respiratory failure in the anti‐synthetase syndrome

机译:他克莫司抢救疗法用于抗合成酶综合征的严重呼吸衰竭

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AbstractInterstitial lung disease (ILD) is the major determinant of morbidity and mortality in the anti-synthetase syndrome (ASS). The therapeutic efficacy of corticosteroids for the ILD component is limited; hence, additional immunosuppressive and immunomodulatory therapies have been tried with a modicum of success in recent years. Tacrolimus, a calcineurin inhibitor, is one potential therapy. We describe four consecutive patients with ASS whom we treated with tacrolimus at a quaternary referral hospital in 2009–2013. All four patients had significant ILD, three had severe and progressive ILD, and two had been referred for consideration of lung transplantation. Tacrolimus use was associated with improvement in ILD in all four patients with a mean follow-up of 3 years. Our case series adds further evidence to support the use of tacrolimus as salvage therapy for severe respiratory failure due to ILD in ASS, which may be associated with a dramatic and enduring response.
机译:摘要间质性肺病(ILD)是抗合成酶综合征(ASS)发病率和死亡率的主要决定因素。皮质类固醇对ILD成分的治疗效果有限;因此,近年来尝试了其他的免疫抑制和免疫调节疗法,并取得了一定的成功。他克莫司,一种钙调神经磷酸酶抑制剂,是一种潜在的治疗方法。我们描述了2009-2013年间在四级转诊医院接受他克莫司治疗的连续四例ASS患者。所有4例患者均患有严重的ILD,3例患有严重和进行性ILD,其中2例已考虑进行肺移植。他克莫司的使用与所有4例患者的ILD改善相关,平均随访3年。我们的病例系列进一步增加了证据支持他克莫司作为挽救疗法用于ASS中ILD引起的严重呼吸衰竭,这可能与剧烈而持久的反应有关。

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