HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Circulating fibrocytes as biomarker of prognosis in Hermansky-Pudlak syndrome.

AbstractRATIONALE:
The rate of progression of most interstitial lung diseases (ILD) is unpredictable. Fibrocytes are circulating bone marrow-derived cells that have been implicated in the pathogenesis of lung fibrosis. Hermansky-Pudlak syndrome (HPS), a genetic cause of ILD in early adulthood, allows for study of biomarkers of ILD in a homogeneous population at near-certain risk of developing fibrotic lung disease.
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.
AuthorsAaron 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
JournalAmerican 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
PMID25347450 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)
Chemical References
  • Biomarkers
  • CXCR4 protein, human
  • Receptors, CXCR4
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

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: