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抗合成酶综合征继发危及生命的呼吸衰竭,需体外膜肺氧合治疗。

Life-threatening respiratory failure requiring extra-corporeal membrane oxygenation secondary to the anti-synthetase syndrome.

作者信息

Sampson Caroline, Taylor Jennifer, Dyson Luke, Hassanein Mostafa

机构信息

Department of Anaesthesia and Critical Care, University Hospitals of Leicester NHS Trust, Leicester, UK.

Department of Anaesthesia, Kettering General Hospital, Kettering, UK.

出版信息

J Intensive Care Soc. 2021 Feb;22(1):83-87. doi: 10.1177/1751143719840260. Epub 2019 Apr 17.

Abstract

Veno-venous extra-corporeal membrane oxygenation (VV ECMO) provides support in severe acute respiratory failure (SARF) refractory to maximal conventional ventilatory support. ECMO does not treat the lungs per se, but allows time for the underlying condition to reverse or resolve. Common indications include acute respiratory distress syndrome (ARDS) and life-threatening asthma. On occasion, rarer conditions causing respiratory failure are diagnosed during ECMO support. The anti-synthetase syndrome (ASS) comprises of a group of conditions characterised by the presence of anti-aminoacyl-tRNA-synthetase antibodies with one or more of interstitial lung disease, inflammatory myositis and/or arthritis. Mainstay of management is immunosuppression. Here, we present two patients requiring ECMO support for SARF, whose respiratory function failed to respond to usual treatment for their assumed pneumonia. Both showed a rapid improvement in respiratory function and oxygenation once immunosuppressive therapy was instigated. Further testing revealed anti-synthetase antibodies, therefore both went on to receive further immunosuppression and an ultimately good outcome. Despite life-threatening respiratory failure, VV ECMO support allowed time for stabilisation, diagnosis and treatment. Outcomes in acute inflammatory interstitial pneumonitis are improved if immunosuppressive treatment is initiated as soon as possible. Our experience with these two cases have led to an institutional change in practice to send an urgent auto-antibody screen (including extractable nuclear antigen panel) on admission for all our SARF patients.

摘要

静脉-静脉体外膜肺氧合(VV ECMO)为严重急性呼吸衰竭(SARF)提供支持,这类呼吸衰竭对最大程度的传统通气支持无效。ECMO本身并不治疗肺部疾病,而是为潜在疾病的逆转或解决争取时间。常见适应症包括急性呼吸窘迫综合征(ARDS)和危及生命的哮喘。有时,在ECMO支持期间会诊断出导致呼吸衰竭的罕见疾病。抗合成酶综合征(ASS)由一组疾病组成,其特征是存在抗氨酰tRNA合成酶抗体,并伴有间质性肺病、炎性肌炎和/或关节炎中的一种或多种。治疗的主要方法是免疫抑制。在此,我们报告两名因SARF需要ECMO支持的患者,他们的呼吸功能对假定的肺炎常规治疗无反应。一旦开始免疫抑制治疗,两人的呼吸功能和氧合情况均迅速改善。进一步检测发现了抗合成酶抗体,因此两人均继续接受进一步的免疫抑制治疗,最终预后良好。尽管存在危及生命的呼吸衰竭,但VV ECMO支持为病情稳定、诊断和治疗争取了时间。如果尽早开始免疫抑制治疗,急性炎症性间质性肺炎的预后会得到改善。我们对这两个病例的经验促使我们机构在实践中做出改变,即对所有SARF患者入院时进行紧急自身抗体筛查(包括可提取核抗原检测)。

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本文引用的文献

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