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 sporadic and genetic subtypes of pulmonary fibrosis. Thus far, one hundred one 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, lung tissue) are being performed to study and compare genetic polymorphisms, gene and protein expression, and clinical findings (e.g., lung physiology, chest radiography, 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 University's Pulmonary Center, will analyze clinical biomarkers of fibrosis. Collaborations with Dr. Jonathan Orens, Johns Hopkins University, and Dr. Steven D. Nathan, INOVA Transplant Center, have been established to procure and study lung tissue specimens.
| Gahl, William A; Brantly, Mark; Troendle, James et al. (2002) Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome. Mol Genet Metab 76:234-42 |
| Gochuico, B R (2001) Potential pathogenesis and clinical aspects of pulmonary fibrosis associated with rheumatoid arthritis. Am J Med Sci 321:83-8 |
