Dissociation of systemic and mucosal autoimmunity in cystic fibrosis.

ABSTRACT:

Background

Pseudomonas aeruginosa accounts for ~80% of cystic fibrosis (CF) airway infection. It shows a remarkable correlation with presence of autoantibody to bactericidal/permeability-increasing protein (BPI), which is not understood. In this study, we sought to better understand the characteristics of systemic and mucosal autoimmunity and their relation to humoral immunity to P. aeruginosa.

Methods

Antibody titers and isotypes to BPI and P. aeruginosa were characterized in sera and bronchoalveolar lavage (BAL) of adult and pediatric CF patients (n = 131), by ELISA and/or immunoblot.

Results

Serum BPI autoantibodies were common (~43%) in adult while rare (≪5%) in pediatric (≤18 yrs) CF patients. Serum BPI IgG autoantibodies were of high avidity and strongly correla

SUBMITTER: Theprungsirikul J 

PROVIDER: S-EPMC10377741 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature