The lung in inborn errors of immunity: From clinical disease patterns to molecular pathogenesis.

ABSTRACT: In addition to being a vital organ for gas exchange, the lung is a crucial immune organ continuously exposed to the external environment. Genetic defects that impair immune function, called inborn errors of immunity (IEI), often have lung disease as the initial and/or primary manifestation. Common types of lung disease seen in IEI include infectious complications and a diverse group of diffuse interstitial lung diseases. Although lung damage in IEI has been historically ascribed to recurrent infections, contributions from potentially targetable autoimmune and inflammatory pathways are now increasingly recognized. This article provides a practical guide to identifying the diverse pulmonary disease patterns in IEI based on lung imaging and respiratory manifestations, and integrates this clin

SUBMITTER: Gutierrez MJ 

PROVIDER: S-EPMC9826631 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature