Translational research projects studying interstitial lung disease have been initiated to examine the pathogenesis of pulmonary fibrosis within subpopulations of patients, focussing on genetic effects. Diseases of interest include familial idiopathic pulmonary fibrosis and lung fibrosis associated with rheumatoid arthritis. Thus far, thirty-six patients have been enrolled in three clinical research protocols (i.e., 99-HL-0056, 99-HL-0068, 99-HL-0069). Analyses of patient-derived specimens (e.g., genomic DNA, serum, bronchoalveolar lavage cells and fluid) are being performed to correlate genetic polymorphisms and large scale gene expression with clinical findings (e.g., lung physiology, chest radiography, diethylenetriamine pentaacetate [DTPA] lung clearance scan).These pulmonary fibrosis protocols have generated collaborations with several intramural and extramural investigators. High-resolution computed tomography and aerosolized DTPA lung clearance scan findings are being examined by NIH Clinical Center researchers, Drs. Nilo Avila and Clara Chen, respectively. Intramural NHLBI pathologists, Drs. William Travis and Victor Ferrans, are studying histopathologic aspects of interstitial lung disease. Dr. Ronald Goldstein, Professor of Medicine from Boston Universitys Pulmonary Center, will analyze clinical biomarkers of fibrosis. - interstitial lung disease, pulmonary fibrosis, genetic polymorphism, rheumatoid arthritis, collagen vascular disease, lung - Human Subjects
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