Alpha-1 Antitrypsin for Cystic Fibrosis Complicated by Severe Cytokinemic COVID-19

Overview

Journal J Cyst Fibros

Publisher Elsevier

Specialty Pulmonary Medicine

Date 2020 Dec 8

PMID 33288475

Citations 12

Authors

Oliver J McElvaney

Eoin OConnor

Natalie L McEvoy

Daniel D Fraughan

Jennifer Clarke

Oisin F McElvaney

Cedric Gunaratnam

James ORourke

Gerard F Curley

Noel G McElvaney

Affiliations

Soon will be listed here.

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.

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