Blood transcriptomic predicts progression of pulmonary fibrosis and associates natural killer cells.
File(s)FVC paper_marked.R2.Jan22.docx (121.32 KB)
Accepted version
Author(s)
Type
Journal Article
Abstract
Rationale: Disease activity in idiopathic pulmonary fibrosis (IPF) remains highly variable, poorly understood, and difficult to predict. Objective: To identify a predictor using short-term longitudinal changes in gene-expression that forecasts future forced vital capacity (FVC) decline and to characterize involved pathways and cell types. Methods: Seventy-four patients from Correlating Outcomes with biochemical Markers to Estimate Time-progression in IPF (COMET) cohort were dichotomized as progressors (≥10% FVC decline) or stable. Blood gene-expression changes within individuals were calculated between baseline and 4 months, and regressed with future FVC status, allowing determination of expression variations, sample size, and statistical power. Pathway analyses were conducted to predict downstream effects and identify new targets. An FVC-predictor for progression was constructed in COMET and validated using independent cohorts. Peripheral blood mononuclear single-cell RNA-seq (PBMC scRNA-seq) data from healthy controls were used as references to characterize cell type compositions from bulk PBMC RNA-seq data that were associated with FVC decline. Results: The longitudinal model reduced gene-expression variations within stable and progressor groups, resulting in increased statistical power when compared to a cross-sectional model. The FVC-predictor for progression anticipated patients with future FVC decline with 78% sensitivity and 86% specificity across independent IPF cohorts. Pattern recognition receptor pathways and mTOR pathways were down- and up-regulated, respectively. Cellular deconvolution using scRNA-seq data identified natural killer (NK) cells as significantly correlated with progression. Conclusions: Serial transcriptomic change predicts future FVC decline. Analysis of cell types involved in the progressor signature supports the novel involvement of NK cells in IPF progression.
Date Issued
2021-03-09
Date Acceptance
2021-03-08
Citation
American Journal of Respiratory and Critical Care Medicine, 2021, 204 (2), pp.197-208
ISSN
1073-449X
Publisher
American Thoracic Society
Start Page
197
End Page
208
Journal / Book Title
American Journal of Respiratory and Critical Care Medicine
Volume
204
Issue
2
Copyright Statement
© 2021 by the American Thoracic Society
Sponsor
Action for Pulmonary Fibrosis
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/33689671
Grant Number
n/a
Subjects
Cell type composition deconvolution
Idiopathic pulmonary fibrosis
Longitudinal changes of blood gene expression
Multigene predictor for progression
Relative decline of forced vital capacity
Publication Status
Published
Coverage Spatial
United States
Date Publish Online
2021-03-09