Enhanced Bruton's tyrosine kinase in B-cells and autoreactive IgA in patients with idiopathic pulmonary fibrosis.

ABSTRACT:

Rationale

Idiopathic Pulmonary Fibrosis (IPF) is thought to be triggered by repeated alveolar epithelial cell injury. Current evidence suggests that aberrant immune activation may contribute. However, the role of B-cell activation remains unclear. We determined the phenotype and activation status of B-cell subsets and evaluated the contribution of activated B-cells to the development of lung fibrosis both in humans and in mice.

Methods

B-cells in blood, mediastinal lymph node, and lung single-cell suspensions of IPF patients and healthy controls (HC) were characterized using 14-color flow cytometry. Mice were exposed to bleomycin to provoke pulmonary fibrosis.

Results

More IgA⁺ memory B-cells and plasmablasts were found in blood (n = 27) and lungs (n = 11)

SUBMITTER: Heukels P 

PROVIDER: S-EPMC6814043 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature