Researchers revealed that computed tomography (CT) imaging, when combined with pulmonary function testing (PFT) and developed into a composite score, offered a new, more sensitive way of determining treatment effects in young patients with cystic fibrosis. The group consisted of young persons who had suffered little or no loss of pulmonary function from their illness. The investigators developed the new approach while assessing a new drug for cystic fibrosis (CF) in 21 young patients over 1 year. The composite CT/PFT score that they developed during the trial combined aspects of functional assessment (the PFT included percent of predicted lung function testing) plus structural analysis of the lung with high resolution computed tomography. They found that their new technique offered them a greater ability to detect changes produced by therapeutic intervention than did the individual components alone. (CF is an inherited disease resulting from two defective genes. Starting at birth, certain glands begin to produce abnormal secretions in nearly all of the glands that secret into a duct (exocrine glands). The lungs are one of the main organs affected.) The authors point out that their findings suggest the potential advantages of using the CT/pulmonary function composite scoring in future clinical trials. However, they note that their conclusions were obtained in a small sample (21 subjects over 1 year) who had CF with minimal decreased pulmonary function and lung disease as determined by high resolution computerized scoring. They urged tests with a larger sample of children and adults who had more extensive CF lung disease. The study appears in the first issue for September 2003 of the American Thoracic Society's peer-reviewed American Journal of Respiratory and Critical Care Medicine.
NEW TEST DEVELOPED TO DETERMINE INCREASED MORTALITY RISK IN INTERSTITIAL PNEUMONIA PATIENTS
A decline in pulmonary function as determined by a 10 percent or more decrease in the forced vital capacity test over a 6-month period was significantly associated with an increased risk of death for patients with idiopathic interstitial pneumonia. Researchers retrospectively examined data from 80 patients with usual interstitial pneumonia and 29 patients with nonspecific interstitial pneumonia. For patients with usual interstitial pneumonia (the most prevalent kind involving fibrosis), a change in forced vital capacity (FVC) over a 6- month period was the best physiologic predictor of mortality. (FVC measures the maximum volume of air that can be exhaled after taking a full breath.) Usual interstitial pneumonia or idiopathic (of unknown cause) pulmonary fibrosis results from an abnormal accumulation of inflammatory cells in lung tissue. During the early stages of the disease, protein-rich fluid and white cells accumulate in the lungs' air sacs, causing inflammation. When the inflammation persists, the fluid can solidify and fibrosis (scarring) replaces lung tissue. That occurrence impairs gas exchange in the lung. In this study, the median survival time for the patients was 5.81 years. The research results appear in the first issue for September 2003 of the American Thoracic Society's peer-reviewed American Journal of Respiratory and Critical Care Medicine. In an editorial in the same issue, two experts discuss the topic of predicting survival in idiopathic interstitial pneumonia. They point out that other recent studies in idiopathic pulmonary fibrosis reveal that the mean survival time is closer to 2 to 3 years than to 5 years. They note that the relative inefficiency of current therapies against pulmonary fibrosis lead to variable rates of disease progression among individual patients. They stressed that developing an improved understanding of the predictors of disease progression and survival was critical in helping patients and clinicians plan for the future, as well as in helping them to determine the optimal time for therapeutic intervention or for lung transplantation.
For the complete text of these articles, please see the American Thoracic Society Online Web Site at http://www.atsjournals.org. For either contact information or to request a complimentary journalist subscription to ATS journals online, or if you would like to add your name to the Society's twice monthly journal news e-mail list, contact Cathy Carlomagno at 212-315-6442, or by e-mail at ccarlomagno@thoracic.org.
Journal
American Journal of Respiratory and Critical Care Medicine