Abstract
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.
Keywords: Alpha-1 antitrypsin; Anti-inflammatory; COVID-19; Coronavirus; Cystic fibrosis; Cytokinemia; Inflammation; Interleukin-1β; Interleukin-6; Neutrophil elastase.
【저자키워드】 COVID-19, Inflammation, coronavirus, Anti-inflammatory, interleukin-6, alpha-1 antitrypsin, cystic fibrosis, interleukin-1β, Cytokinemia, Neutrophil elastase., 【초록키워드】 therapy, severe COVID-19, Anti-inflammatory, IL-6, neutrophil, interleukin-6, lung, fibrosis, airway, Clinical course, interleukin, therapeutic, Patient, IL-8, Alpha, plasma, Inflammatory marker, cystic fibrosis, receptor, Neutrophil elastase, interleukin-1β, TNF, IL-1β, dose, Inflammatory, lung transplantation, systemic, Support, Radiographic, baseline levels, exploration, antitrypsin, parameter, Administered, decrease, Level, interval, baseline, Cystic, were assessed, 【제목키워드】 cystic fibrosis,