Exacerbations of Lung Disease in Alpha-1 Antitrypsin Deficiency

Link to article at PubMed

Chronic Obstr Pulm Dis. 2020 Nov 25. doi: 10.15326/jcopdf.2020.0173. Online ahead of print.


Alpha-1 antitrypsin deficiency (AATD) is an important risk factor for development of chronic obstructive pulmonary disease (COPD). Patients with AATD classically develop a different pattern of lung disease from those with usual COPD, decline faster and exhibit a range of differences in pathogenesis, all of which may be relevant to phenotype and/or impact of exacerbations. There are a number of definitions of exacerbation, with the main features being worsening of symptoms over at least 2 days, which may be associated with a change in treatment. In this article we review the literature surrounding exacerbations in AATD, focussing in particular on ways in which they may differ from such events in usual COPD, and the potential impact on clinical management.

PMID:33238089 | DOI:10.15326/jcopdf.2020.0173

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