Jenny Y Chen1, Megan Griffiths2, Jun Yang2, Melanie K Nies2, Rachel L Damico3, Catherine E Simpson3, R Dhananjay Vaidya4, Stephanie Brandal2, D Dunbar Ivy5, Eric D Austin6, William C Nichols7, Michael W Pauciulo7, Katie Lutz7, Erika B Rosenzweig8, Russel Hirsch9, Delphine Yung10, Allen D Everett11. 1. Johns Hopkins University School of Medicine, Baltimore, MD. 2. Division of Pediatric Cardiology, Department of Pediatrics, Johns Hopkins University, Baltimore, MD. 3. Department of Anesthesia and Critical Care Medicine, Johns Hopkins University, Baltimore, MD. 4. Department of Internal Medicine, Johns Hopkins University, Baltimore, MD. 5. Department of Pediatric Cardiology, Children's Hospital Colorado, Denver, CO. 6. Division of Allergy, Immunology, and Pulmonary Medicine, Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN. 7. Division of Human Genetics, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH. 8. Division of Pediatric Cardiology, Department of Pediatrics, Columbia University, New York City, NY. 9. Division of Pediatric Cardiology, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH. 10. Division of Pediatric Cardiology, Department of Pediatrics, University of Washington, Seattle, WA. 11. Division of Pediatric Cardiology, Department of Pediatrics, Johns Hopkins University, Baltimore, MD. Electronic address: aeveret3@jhmi.edu.
Abstract
OBJECTIVE: To assess whether circulating interleukin-6 (IL-6) is associated with measures of disease severity and clinical worsening in pediatric pulmonary arterial hypertension (PAH). STUDY DESIGN: IL-6 was measured by enzyme-linked immunosorbent assay in serum samples from a cross-sectional cohort from the National Heart, Lung, and Blood Institute Pulmonary Arterial Hypertension Biobank (n = 175) and a longitudinal cohort from Children's Hospital Colorado (CHC) (n = 61). Associations between IL-6, disease severity, and outcomes were studied with regression and Kaplan-Meier analysis. RESULTS: In analyses adjusted for age and sex, each log-unit greater IL-6 was significantly associated in the Pulmonary Arterial Hypertension Biobank cohort with greater pulmonary vascular resistance indices, lower odds of having idiopathic PAH or treatment with prostacyclin, and greater odds of having PAH associated with a repaired congenital shunt. In the CHC cohort, each log-unit greater IL-6 was significantly associated with greater mean pulmonary arterial pressure over time. Kaplan-Meier analysis in the CHC cohort revealed that IL-6 was significantly associated with clinical worsening (a composite score of mortality, transplant, or palliative surgery) (P = .037). CONCLUSIONS: IL-6 was significantly associated with worse hemodynamics at baseline and over time and may be associated with clinical worsening. IL-6 may provide a less-invasive method for disease monitoring and prognosis in pediatric PAH as well as a potential therapeutic target.
OBJECTIVE: To assess whether circulating interleukin-6 (IL-6) is associated with measures of disease severity and clinical worsening in pediatric pulmonary arterial hypertension (PAH). STUDY DESIGN:IL-6 was measured by enzyme-linked immunosorbent assay in serum samples from a cross-sectional cohort from the National Heart, Lung, and Blood Institute Pulmonary Arterial Hypertension Biobank (n = 175) and a longitudinal cohort from Children's Hospital Colorado (CHC) (n = 61). Associations between IL-6, disease severity, and outcomes were studied with regression and Kaplan-Meier analysis. RESULTS: In analyses adjusted for age and sex, each log-unit greater IL-6 was significantly associated in the Pulmonary Arterial Hypertension Biobank cohort with greater pulmonary vascular resistance indices, lower odds of having idiopathic PAH or treatment with prostacyclin, and greater odds of having PAH associated with a repaired congenital shunt. In the CHC cohort, each log-unit greater IL-6 was significantly associated with greater mean pulmonary arterial pressure over time. Kaplan-Meier analysis in the CHC cohort revealed that IL-6 was significantly associated with clinical worsening (a composite score of mortality, transplant, or palliative surgery) (P = .037). CONCLUSIONS:IL-6 was significantly associated with worse hemodynamics at baseline and over time and may be associated with clinical worsening. IL-6 may provide a less-invasive method for disease monitoring and prognosis in pediatric PAH as well as a potential therapeutic target.
Authors: Michal Schäfer; Uyen Truong; Lorna P Browne; Gareth J Morgan; Michael Ross; Richard Ing; Kendall S Hunter; Vitaly O Kheyfets; Steven H Abman; D Dunbar Ivy; Neil Wilson Journal: Pediatr Cardiol Date: 2017-10-17 Impact factor: 1.655
Authors: Elaine Soon; Alan M Holmes; Carmen M Treacy; Natalie J Doughty; Laura Southgate; Rajiv D Machado; Richard C Trembath; Simon Jennings; Lucy Barker; Paul Nicklin; Christoph Walker; David C Budd; Joanna Pepke-Zaba; Nicholas W Morrell Journal: Circulation Date: 2010-08-16 Impact factor: 29.690
Authors: Erika B Rosenzweig; Steven H Abman; Ian Adatia; Maurice Beghetti; Damien Bonnet; Sheila Haworth; D Dunbar Ivy; Rolf M F Berger Journal: Eur Respir J Date: 2019-01-24 Impact factor: 16.671
Authors: Caroline M Daly; Megan Griffiths; Catherine E Simpson; Jun Yang; Rachel L Damico; R Dhananjay Vaidya; Monica Williams; Stephanie Brandal; Pei-Ni Jone; Cassandra Polsen; D Dunbar Ivy; Eric D Austin; William C Nichols; Michael W Pauciulo; Katie Lutz; Melanie K Nies; Erika B Rosenzweig; Russel Hirsch; Delphine Yung; Allen D Everett Journal: J Am Heart Assoc Date: 2021-10-08 Impact factor: 6.106