Circulating Fibrocytes as Biomarker of Prognosis in Hermansky-Pudlak Syndrome
| العنوان: | Circulating Fibrocytes as Biomarker of Prognosis in Hermansky-Pudlak Syndrome |
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| المؤلفون: | Borna Mehrad, Marie D. Burdick, Jae K. Lee, Robert M. Strieter, Thomas C. Markello, Youngchul Kim, Roxanne Fischer, William A. Gahl, Bernadette R. Gochuico, Aaron T. Trimble |
| المصدر: | American Journal of Respiratory and Critical Care Medicine. 190:1395-1401 |
| بيانات النشر: | American Thoracic Society, 2014. |
| سنة النشر: | 2014 |
| مصطلحات موضوعية: | Pulmonary and Respiratory Medicine, Pathology, medicine.medical_specialty, education.field_of_study, Lung, business.industry, Population, Case-control study, respiratory system, Critical Care and Intensive Care Medicine, CXCR4, respiratory tract diseases, Pathogenesis, medicine.anatomical_structure, Fibrocyte, Immunology, medicine, Biomarker (medicine), education, business, Survival analysis |
| الوصف: | 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. 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. |
| تدمد: | 1535-4970 1073-449X |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::05cee66dc7e63ec055c646e28597e64e https://doi.org/10.1164/rccm.201407-1287oc |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi...........05cee66dc7e63ec055c646e28597e64e |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15354970 1073449X |
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