用于合并严重细胞因子血症 COVID-19 的囊性纤维化的 α-1 抗胰蛋白酶。
Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.
机构信息
Department of Medicine, Royal College of Surgeons in Ireland, Dublin, Ireland; Beaumont Hospital, Dublin, Ireland.
Beaumont Hospital, Dublin, Ireland.
出版信息
J Cyst Fibros. 2021 Jan;20(1):31-35. doi: 10.1016/j.jcf.2020.11.012. Epub 2020 Nov 20.
BACKGROUND
The clinical course of severe COVID-19 in cystic fibrosis (CF) is incompletely understood. We describe the use of alpha-1 antitrypsin (AAT) as a salvage therapy in a critically unwell patient with CF (PWCF) who developed COVID-19 while awaiting lung transplantation.
METHODS
IV AAT was administered at 120 mg/kg/week for 4 consecutive weeks. Levels of interleukin (IL)-1β, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1β, IL-6, IL-8 and neutrophil elastase (NE) activity measured in airway secretions. Levels were compared to baseline and historic severe exacerbation measurements.
RESULTS
Systemic and airway inflammatory markers were increased compared to both prior exacerbation and baseline levels, in particular IL-6, IL-1β and NE activity. Following each AAT dose, rapid decreases in each inflammatory parameter were observed. These were matched by marked clinical and radiographic improvement.
CONCLUSIONS
The results support further investigation of AAT as a COVID-19 therapeutic, and re-exploration of its use in CF.
背景
囊性纤维化 (CF) 患者中严重 COVID-19 的临床病程尚不完全清楚。我们描述了在等待肺移植时患有 CF（PWCF）并发生 COVID-19 的情况下，使用α-1 抗胰蛋白酶 (AAT) 作为挽救疗法的情况。
方法
每周给予 IV AAT 120mg/kg，连续 4 周。定期评估血浆中白细胞介素 (IL)-1β、IL-6、IL-8 和可溶性 TNF 受体 1 (sTNFR1) 的水平，并测量气道分泌物中的 IL-1β、IL-6、IL-8 和中性粒细胞弹性蛋白酶 (NE) 活性。将这些水平与基线和既往严重加重的测量值进行比较。
结果
与既往加重和基线水平相比，全身和气道炎症标志物均升高，尤其是 IL-6、IL-1β 和 NE 活性。每次给予 AAT 后，观察到每个炎症参数迅速下降。这与明显的临床和影像学改善相匹配。
结论
结果支持进一步研究 AAT 作为 COVID-19 治疗药物，并重新探索其在 CF 中的应用。
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