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Cells, collagen and idiopathic pulmonary fibrosis

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Abstract

Idiopathic pulmonary fibrosis (IPF) is a usually fatal disorder of lung with clearly defined clinical, roentgenographic, physiologic, morphologic, scintigraphic and bronchoalveolar lavage features. Current concepts of the pathogenesis of this disorder suggest a central role for a chronic alveolitis in causing changes in parenchymal cell populations and derangements in interstitial collagen. Of the many inflammatory and immune effector cells comprising the alveolitis of IPF, it is likely that the neutrophil is the most important mediator of parenchymal damage. To follow the status of lung neutrophils in patients with this disease, two methods have been utilized. Both gallium-67 scanning and bronchoalveolar lavage quantitate the extent of the alveolitis and can be used to stage and follow these patients. The treatment of IPF remains controversial, but it is likely that corticosteroids reduce the alveolitis and prolong the lifespan of these patients.

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Authors and Affiliations

  1. Pulmonary Branch and Pathology Branch, National Heart, Lung, and Blood Institute, and Department of Nuclear Medicine, Clinical Center, National Institutes of Health, Bethesda, Maryland, USA

    Ronald G. Crystal M. D., Jack D. Fulmer, Bruce J. Baum, John Bernardo, Kathryn H. Bradley, Sally D. Bruel, Norton A. Elson, Gerald A. Fells, Victor J. Ferrans, James E. Gadek, Gary W. Hunninghake, Oichi Kawanami, Jeffrey A. Kelman, Bruce R. Line, John A. McDonald, Bryon D. McLees, William C. Roberts, David M. Rosenberg, Paul Tolstoshev, Edwyna Von Gal & Steven E. Weinberger

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  1. Ronald G. Crystal M. D.
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  2. Jack D. Fulmer
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  3. Bruce J. Baum
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  4. John Bernardo
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  5. Kathryn H. Bradley
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  6. Sally D. Bruel
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  7. Norton A. Elson
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  8. Gerald A. Fells
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  9. Victor J. Ferrans
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  10. James E. Gadek
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  11. Gary W. Hunninghake
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  12. Oichi Kawanami
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  13. Jeffrey A. Kelman
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  14. Bruce R. Line
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  15. John A. McDonald
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  16. Bryon D. McLees
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  17. William C. Roberts
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  18. David M. Rosenberg
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Crystal, R.G., Fulmer, J.D., Baum, B.J. et al. Cells, collagen and idiopathic pulmonary fibrosis. Lung 155, 199–224 (1978). https://doi.org/10.1007/BF02730694

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  • DOI: https://doi.org/10.1007/BF02730694

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