Circulating fibrocytes as biomarker of prognosis in Hermansky-Pudlak syndrome

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

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Rationale: 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.

Conclusions: CXCR41 fibrocyte concentration may be useful as a biomarker for outcome of ILD in subjects with HPS.

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 CXCR41 fibrocyte concentration were strongly associated with death from ILD.

Original languageEnglish (US)
Pages (from-to)1395-1401
Number of pages7
JournalAmerican journal of respiratory and critical care medicine
Issue number12
StatePublished - Dec 15 2014
Externally publishedYes


  • CXCR4 receptors
  • Chemokines
  • Interstitial lung diseases
  • Stem cells

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


Dive into the research topics of 'Circulating fibrocytes as biomarker of prognosis in Hermansky-Pudlak syndrome'. Together they form a unique fingerprint.

Cite this