Abstract | RATIONALE: OBJECTIVES: To test the hypothesis that, in subjects with HPS, the number or phenotype of circulating fibrocytes predicts progression and outcome of ILD. METHODS: We measured circulating fibrocyte counts and chemokine levels in a cohort of subjects with HPS and healthy control subjects and correlated the results to disease outcome. MEASUREMENTS AND MAIN RESULTS: In a cross-sectional analysis, peripheral blood fibrocyte concentrations were markedly elevated in a subset of subjects with HPS who had ILD but not subjects without lung disease or normal control subjects. The blood concentration of fibrocytes expressing the chemokine receptor CXCR4 correlated significantly with the plasma concentration of the CXCR4 ligand, CXCL12. In a longitudinal study, we found marked episodic elevations in circulating fibrocyte counts over a median follow-up period of 614 days. Elevations in both maximal values and final values of peripheral blood CXCR4(+) fibrocyte concentration were strongly associated with death from ILD. CONCLUSIONS: CXCR4(+) fibrocyte concentration may be useful as a biomarker for outcome of ILD in subjects with HPS.
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Authors | Aaron Trimble, Bernadette R Gochuico, Thomas C Markello, Roxanne Fischer, William A Gahl, Jae K Lee, Youngchul Kim, Marie D Burdick, Robert M Strieter, Borna Mehrad |
Journal | American journal of respiratory and critical care medicine
(Am J Respir Crit Care Med)
Vol. 190
Issue 12
Pg. 1395-401
(Dec 15 2014)
ISSN: 1535-4970 [Electronic] United States |
PMID | 25347450
(Publication Type: Journal Article, Research Support, N.I.H., Extramural)
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Chemical References |
- Biomarkers
- CXCR4 protein, human
- Receptors, CXCR4
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Topics |
- Adult
- Biomarkers
(blood)
- Case-Control Studies
- Cross-Sectional Studies
- Female
- Hermanski-Pudlak Syndrome
(blood, diagnosis, mortality)
- Humans
- Longitudinal Studies
- Male
- Mesenchymal Stem Cells
(physiology)
- Middle Aged
- Prognosis
- Receptors, CXCR4
(blood, physiology)
- Survival Analysis
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