Idiopathic Pulmonary Fibrosis

doi:10.1056/NEJMra003200

Volume 345:517-525		August 16, 2001		Number 7

Idiopathic Pulmonary Fibrosis

Thomas J. Gross, M.D., and Gary W. Hunninghake, M.D.

Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

Full Text

PDF

Purchase this article

Add to Personal Archive

Add to Citation Manager

Notify a Friend

E-mail When Cited

PubMed Citation

Idiopathic pulmonary fibrosis is characterized by radiographicallyevident interstitial infiltrates predominantly affecting thelung bases and by progressive dyspnea and worsening of pulmonaryfunction. No therapy has been clearly shown to prolong survival.¹The current strict definition of idiopathic pulmonary fibrosisprovides a new focus for basic and clinical research that willimprove insight into the pathogenesis of this disorder and stimulatethe development of novel therapies.

Definition

Idiopathic pulmonary fibrosis, also known as cryptogenic fibrosingalveolitis, is one of a family of idiopathic pneumonias sharingthe clinical features of shortness of breath, radiographicallyevident diffuse pulmonary infiltrates, and varying degrees . . . [Full Text of this Article]

Pathogenesis

Diagnosis

Natural History

Therapy

Antiinflammatory Agents

Antifibrotic Agents

Immune Modulators

Lung Transplantation

Summary

Source Information

From the Division of Pulmonary, Critical Care, and Occupational Medicine, Department of Internal Medicine, University of Iowa College of Medicine and Veterans Affairs Medical Center, Iowa City.

Address reprint requests to Dr. Hunninghake at the Division of Pulmonary, Critical Care, and Occupational Medicine, Department of Internal Medicine, Room C33-GH, University of Iowa Health Care, Iowa City, IA 52242, or at gary-hunninghake@uiowa.edu.

References

This article has been cited by other articles:

Behr, J., Ryu, J. H. (2008). Pulmonary hypertension in interstitial lung disease. Eur Respir J 31: 1357-1367 [Abstract] [Full Text]
Lawson, W. E., Crossno, P. F., Polosukhin, V. V., Roldan, J., Cheng, D.-S., Lane, K. B., Blackwell, T. R., Xu, C., Markin, C., Ware, L. B., Miller, G. G., Loyd, J. E., Blackwell, T. S. (2008). Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. Am. J. Physiol. Lung Cell. Mol. Physiol. 294: L1119-L1126 [Abstract] [Full Text]
Milam, J. E., Keshamouni, V. G., Phan, S. H., Hu, B., Gangireddy, S. R., Hogaboam, C. M., Standiford, T. J., Thannickal, V. J., Reddy, R. C. (2008). PPAR-{gamma} agonists inhibit profibrotic phenotypes in human lung fibroblasts and bleomycin-induced pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol. 294: L891-L901 [Abstract] [Full Text]
Rogliani, P., Mura, M., Assunta Porretta, M., Saltini, C. (2008). Review: New perspectives in the treatment of idiopathic pulmonary fibrosis. Therapeutic Advances in Respiratory Disease 2: 75-93 [Abstract]
Clement, A., Eber, E. (2008). Interstitial lung diseases in infants and children. Eur Respir J 31: 658-666 [Abstract] [Full Text]
Myllarniemi, M., Lindholm, P., Ryynanen, M. J., Kliment, C. R., Salmenkivi, K., Keski-Oja, J., Kinnula, V. L., Oury, T. D., Koli, K. (2008). Gremlin-mediated Decrease in Bone Morphogenetic Protein Signaling Promotes Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 177: 321-329 [Abstract] [Full Text]
Kitowska, K., Zakrzewicz, D., Konigshoff, M., Chrobak, I., Grimminger, F., Seeger, W., Bulau, P., Eickelberg, O. (2008). Functional role and species-specific contribution of arginases in pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol. 294: L34-L45 [Abstract] [Full Text]
Azuma, M., Nishioka, Y., Aono, Y., Inayama, M., Makino, H., Kishi, J., Shono, M., Kinoshita, K., Uehara, H., Ogushi, F., Izumi, K., Sone, S. (2007). Role of {alpha}1-Acid Glycoprotein in Therapeutic Antifibrotic Effects of Imatinib with Macrolides in Mice. Am. J. Respir. Crit. Care Med. 176: 1243-1250 [Abstract] [Full Text]
Garcia, C. K., Wright, W. E., Shay, J. W. (2007). Human diseases of telomerase dysfunction: insights into tissue aging. Nucleic Acids Res 35: 7406-7416 [Abstract] [Full Text]
Tzouvelekis, A., Harokopos, V., Paparountas, T., Oikonomou, N., Chatziioannou, A., Vilaras, G., Tsiambas, E., Karameris, A., Bouros, D., Aidinis, V. (2007). Comparative Expression Profiling in Pulmonary Fibrosis Suggests a Role of Hypoxia-inducible Factor-1{alpha} in Disease Pathogenesis. Am. J. Respir. Crit. Care Med. 176: 1108-1119 [Abstract] [Full Text]
Perri, D., Cole, D. E. C., Friedman, O., Piliotis, E., Mintz, S., Adhikari, N. K. J. (2007). Azathioprine and diffuse alveolar haemorrhage: the pharmacogenetics of thiopurine methyltransferase. Eur Respir J 30: 1014-1017 [Abstract] [Full Text]
Kabuyama, Y., Oshima, K., Kitamura, T., Homma, M., Yamaki, J., Munakata, M., Homma, Y. (2007). Involvement of selenoprotein P in the regulation of redox balance and myofibroblast viability in idiopathic pulmonary fibrosis.. GENES CELLS 12: 1235-1244 [Abstract] [Full Text]
Mason, D. P., Brizzio, M. E., Alster, J. M., McNeill, A. M., Murthy, S. C., Budev, M. M., Mehta, A. C., Minai, O. A., Pettersson, G. B., Blackstone, E. H. (2007). Lung Transplantation for Idiopathic Pulmonary Fibrosis. Ann. Thorac. Surg. 84: 1121-1128 [Abstract] [Full Text]
Rosas, I. O., Ren, P., Avila, N. A., Chow, C. K., Franks, T. J., Travis, W. D., McCoy, J. P. Jr., May, R. M., Wu, H.-P., Nguyen, D. M., Arcos-Burgos, M., MacDonald, S. D., Gochuico, B. R. (2007). Early Interstitial Lung Disease in Familial Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 176: 698-705 [Abstract] [Full Text]
Kim, K. K., Chapman, H. A. (2007). Endothelin-1 as Initiator of Epithelial-Mesenchymal Transition: Potential New Role for Endothelin-1 during Pulmonary Fibrosis. Am. J. Respir. Cell Mol. Bio. 37: 1-2 [Full Text]
Tabata, C., Tabata, R., Kadokawa, Y., Hisamori, S., Takahashi, M., Mishima, M., Nakano, T., Kubo, H. (2007). Thalidomide Prevents Bleomycin-Induced Pulmonary Fibrosis in Mice. J. Immunol. 179: 708-714 [Abstract] [Full Text]
Alakhras, M., Decker, P. A., Nadrous, H. F., Collazo-Clavell, M., Ryu, J. H. (2007). Body Mass Index and Mortality in Patients With Idiopathic Pulmonary Fibrosis. Chest 131: 1448-1453 [Abstract] [Full Text]
Tsakiri, K. D., Cronkhite, J. T., Kuan, P. J., Xing, C., Raghu, G., Weissler, J. C., Rosenblatt, R. L., Shay, J. W., Garcia, C. K. (2007). Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proc. Natl. Acad. Sci. USA 104: 7552-7557 [Abstract] [Full Text]
Ishida, Y., Kimura, A., Kondo, T., Hayashi, T., Ueno, M., Takakura, N., Matsushima, K., Mukaida, N. (2007). Essential Roles of the CC Chemokine Ligand 3-CC Chemokine Receptor 5 Axis in Bleomycin-Induced Pulmonary Fibrosis through Regulation of Macrophage and Fibrocyte Infiltration. Am. J. Pathol. 170: 843-854 [Abstract] [Full Text]
Gonzalez, A. V., Le Bellego, F., Ludwig, M. S. (2007). Imbalance of Receptor-Regulated and Inhibitory Smads in Lung Fibroblasts from Bleomycin-Exposed Rats. Am. J. Respir. Cell Mol. Bio. 36: 206-212 [Abstract] [Full Text]
Rudd, R. M, Prescott, R. J, Chalmers, J C, Johnston, I. D A, for the Fibrosing Alveolitis Subcommittee of the R, (2007). British Thoracic Society Study on cryptogenic fibrosing alveolitis: response to treatment and survival. Thorax 62: 62-66 [Abstract] [Full Text]
Tabata, C., Kadokawa, Y., Tabata, R., Takahashi, M., Okoshi, K., Sakai, Y., Mishima, M., Kubo, H. (2006). All-trans-Retinoic Acid Prevents Radiation- or Bleomycin-induced Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 174: 1352-1360 [Abstract] [Full Text]
Gauldie, J., Kolb, M., Ask, K., Martin, G., Bonniaud, P., Warburton, D. (2006). Smad3 Signaling Involved in Pulmonary Fibrosis and Emphysema. Proc Am Thorac Soc 3: 696-702 [Abstract] [Full Text]
Yasuoka, H., Zhou, Z., Pilewski, J. M., Oury, T. D., Choi, A. M.K., Feghali-Bostwick, C. A. (2006). Insulin-Like Growth Factor-Binding Protein-5 Induces Pulmonary Fibrosis and Triggers Mononuclear Cellular Infiltration. Am. J. Pathol. 169: 1633-1642 [Abstract] [Full Text]
Nakagome, K, Dohi, M, Okunishi, K, Tanaka, R, Miyazaki, J, Yamamoto, K (2006). In vivo IL-10 gene delivery attenuates bleomycin induced pulmonary fibrosis by inhibiting the production and activation of TGF-{beta} in the lung. Thorax 61: 886-894 [Abstract] [Full Text]
Lovgren, A. K., Jania, L. A., Hartney, J. M., Parsons, K. K., Audoly, L. P., FitzGerald, G. A., Tilley, S. L., Koller, B. H. (2006). COX-2-derived prostacyclin protects against bleomycin-induced pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol. 291: L144-L156 [Abstract] [Full Text]
Kimura, T., Ishii, Y., Yoh, K., Morishima, Y., Iizuka, T., Kiwamoto, T., Matsuno, Y., Homma, S., Nomura, A., Sakamoto, T., Takahashi, S., Sekizawa, K. (2006). Overexpression of the Transcription Factor GATA-3 Enhances the Development of Pulmonary Fibrosis. Am. J. Pathol. 169: 96-104 [Abstract] [Full Text]
Inayama, M., Nishioka, Y., Azuma, M., Muto, S., Aono, Y., Makino, H., Tani, K., Uehara, H., Izumi, K., Itai, A., Sone, S. (2006). A Novel I{kappa}B Kinase-beta Inhibitor Ameliorates Bleomycin-induced Pulmonary Fibrosis in Mice. Am. J. Respir. Crit. Care Med. 173: 1016-1022 [Abstract] [Full Text]
Yun, A. J., Lee, P. Y., Gerber, A. N. (2006). Integrating systems biology and medical imaging: understanding disease distribution in the lung model.. Am. J. Roentgenol. 186: 925-930 [Abstract] [Full Text]
Prasse, A., Pechkovsky, D. V., Toews, G. B., Jungraithmayr, W., Kollert, F., Goldmann, T., Vollmer, E., Muller-Quernheim, J., Zissel, G. (2006). A Vicious Circle of Alveolar Macrophages and Fibroblasts Perpetuates Pulmonary Fibrosis via CCL18. Am. J. Respir. Crit. Care Med. 173: 781-792 [Abstract] [Full Text]
Budinger, G. R. S., Mutlu, G. M., Eisenbart, J., Fuller, A. C., Bellmeyer, A. A., Baker, C. M., Wilson, M., Ridge, K., Barrett, T. A., Lee, V. Y., Chandel, N. S. (2006). Proapoptotic Bid is required for pulmonary fibrosis. Proc. Natl. Acad. Sci. USA 103: 4604-4609 [Abstract] [Full Text]
Pignatti, P., Brunetti, G., Moretto, D., Yacoub, M.-R., Fiori, M., Balbi, B., Balestrino, A., Cervio, G., Nava, S., Moscato, G. (2006). Role of the Chemokine Receptors CXCR3 and CCR4 in Human Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 173: 310-317 [Abstract] [Full Text]
Visscher, D. W., Myers, J. L. (2006). Histologic spectrum of idiopathic interstitial pneumonias.. Proc Am Thorac Soc 3: 322-329 [Abstract] [Full Text]
Lawson, W. E., Loyd, J. E. (2006). The genetic approach in pulmonary fibrosis: can it provide clues to this complex disease?. Proc Am Thorac Soc 3: 345-349 [Abstract] [Full Text]
Demedts, M., Behr, J., Buhl, R., Costabel, U., Dekhuijzen, R., Jansen, H. M., MacNee, W., Thomeer, M., Wallaert, B., Laurent, F., Nicholson, A. G., Verbeken, E. K., Verschakelen, J., Flower, C. D.R., Capron, F., Petruzzelli, S., De Vuyst, P., van den Bosch, J. M.M., Rodriguez-Becerra, E., Corvasce, G., Lankhorst, I., Sardina, M., Montanari, M., the IFIGENIA Study Group, (2005). High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis.. NEJM 353: 2229-2242 [Abstract] [Full Text]
Hunninghake, G. W. (2005). Antioxidant Therapy for Idiopathic Pulmonary Fibrosis.. NEJM 353: 2285-2287 [Full Text]
Parambil, J. G., Myers, J. L., Ryu, J. H. (2005). Histopathologic Features and Outcome of Patients With Acute Exacerbation of Idiopathic Pulmonary Fibrosis Undergoing Surgical Lung Biopsy. Chest 128: 3310-3315 [Abstract] [Full Text]
Samuel, C. S. (2005). Relaxin: Antifibrotic Properties and Effects in Models of Disease. Clin Med Res 3: 241-249 [Abstract] [Full Text]
Lawson, W. E., Polosukhin, V. V., Stathopoulos, G. T., Zoia, O., Han, W., Lane, K. B., Li, B., Donnelly, E. F., Holburn, G. E., Lewis, K. G., Collins, R. D., Hull, W. M., Glasser, S. W., Whitsett, J. A., Blackwell, T. S. (2005). Increased and Prolonged Pulmonary Fibrosis in Surfactant Protein C-Deficient Mice Following Intratracheal Bleomycin. Am. J. Pathol. 167: 1267-1277 [Abstract] [Full Text]
Bouros, D., Antoniou, K. M. (2005). Current and future therapeutic approaches in idiopathic pulmonary fibrosis. Eur Respir J 26: 693-703 [Abstract] [Full Text]
Nadrous, H. F., Pellikka, P. A., Krowka, M. J., Swanson, K. L., Chaowalit, N., Decker, P. A., Ryu, J. H. (2005). Pulmonary Hypertension in Patients With Idiopathic Pulmonary Fibrosis. Chest 128: 2393-2399 [Abstract] [Full Text]
Pacanowski, M. A, Amsden, G. W (2005). Interferon Gamma-1b in the Treatment of Idiopathic Pulmonary Fibrosis. The Annals of Pharmacotherapy 39: 1678-1686 [Abstract] [Full Text]
Lemay, A.-M., Haston, C. K. (2005). Bleomycin-induced pulmonary fibrosis susceptibility genes in AcB/BcA recombinant congenic mice. Physiol. Genomics 23: 54-61 [Abstract] [Full Text]
Date, H., Tanimoto, Y., Goto, K., Yamadori, I., Aoe, M., Sano, Y., Shimizu, N. (2005). A New Treatment Strategy for Advanced Idiopathic Interstitial Pneumonia*: Living-Donor Lobar Lung Transplantation. Chest 128: 1364-1370 [Abstract] [Full Text]
Kinnula, V. L., Fattman, C. L., Tan, R. J., Oury, T. D. (2005). Oxidative Stress in Pulmonary Fibrosis: A Possible Role for Redox Modulatory Therapy. Am. J. Respir. Crit. Care Med. 172: 417-422 [Abstract] [Full Text]
Nakatani-Okuda, A., Ueda, H., Kashiwamura, S.-i., Sekiyama, A., Kubota, A., Fujita, Y., Adachi, S., Tsuji, Y., Tanizawa, T., Okamura, H. (2005). Protection against bleomycin-induced lung injury by IL-18 in mice. Am. J. Physiol. Lung Cell. Mol. Physiol. 289: L280-L287 [Abstract] [Full Text]
Bajwa, E. K., Ayas, N. T., Schulzer, M., Mak, E., Ryu, J. H., Malhotra, A. (2005). Interferon-{gamma}1b Therapy in Idiopathic Pulmonary Fibrosis: A Metaanalysis. Chest 128: 203-206 [Abstract] [Full Text]
Wasswa-Kintu, S, Gan, W Q, Man, S F P, Pare, P D, Sin, D D (2005). Relationship between reduced forced expiratory volume in one second and the risk of lung cancer: a systematic review and meta-analysis. Thorax 60: 570-575 [Abstract] [Full Text]
Nishiyama, O., Taniguchi, H., Kondoh, Y., Kimura, T., Ogawa, T., Watanabe, F., Arizono, S. (2005). Quadriceps Weakness Is Related to Exercise Capacity in Idiopathic Pulmonary Fibrosis. Chest 127: 2028-2033 [Abstract] [Full Text]
Haston, C K, Tomko, T G, Godin, N, Kerckhoff, L, Hallett, M T (2005). Murine candidate bleomycin induced pulmonary fibrosis susceptibility genes identified by gene expression and sequence analysis of linkage regions. J. Med. Genet. 42: 464-473 [Abstract] [Full Text]
Gharaee-Kermani, M., Hatano, K., Nozaki, Y., Phan, S. H. (2005). Gender-Based Differences in Bleomycin-Induced Pulmonary Fibrosis. Am. J. Pathol. 166: 1593-1606 [Abstract] [Full Text]
Aono, Y., Nishioka, Y., Inayama, M., Ugai, M., Kishi, J., Uehara, H., Izumi, K., Sone, S. (2005). Imatinib as a Novel Antifibrotic Agent in Bleomycin-induced Pulmonary Fibrosis in Mice. Am. J. Respir. Crit. Care Med. 171: 1279-1285 [Abstract] [Full Text]
Abdollahi, A., Li, M., Ping, G., Plathow, C., Domhan, S., Kiessling, F., Lee, L. B., McMahon, G., Grone, H.-J., Lipson, K. E., Huber, P. E. (2005). Inhibition of platelet-derived growth factor signaling attenuates pulmonary fibrosis. J. Exp. Med. 201: 925-935 [Abstract] [Full Text]
Meyer, D. M., Edwards, L. B., Torres, F., Jessen, M. E., Novick, R. J. (2005). Impact of Recipient Age and Procedure Type on Survival After Lung Transplantation for Pulmonary Fibrosis. Ann. Thorac. Surg. 79: 950-957 [Abstract] [Full Text]
Genovese, T., Cuzzocrea, S., Di Paola, R., Mazzon, E., Mastruzzo, C., Catalano, P., Sortino, M., Crimi, N., Caputi, A. P., Thiemermann, C., Vancheri, C. (2005). Effect of rosiglitazone and 15-deoxy-{Delta}12,14-prostaglandin J2 on bleomycin-induced lung injury. Eur Respir J 25: 225-234 [Abstract] [Full Text]
Norris, A. J., Naydan, D. K., Wilson, D. W. (2005). Interstitial Lung Disease in West Highland White Terriers. Vet Pathol 42: 35-41 [Abstract] [Full Text]
Murakami, S., Nagaya, N., Itoh, T., Fujii, T., Iwase, T., Hamada, K., Kimura, H., Kangawa, K. (2004). C-type natriuretic peptide attenuates bleomycin-induced pulmonary fibrosis in mice. Am. J. Physiol. Lung Cell. Mol. Physiol. 287: L1172-L1177 [Abstract] [Full Text]
Hodges, R. J., Jenkins, R. G., Wheeler-Jones, C. P.D., Copeman, D. M., Bottoms, S. E., Bellingan, G. J., Nanthakumar, C. B., Laurent, G. J., Hart, S. L., Foster, M. L., McAnulty, R. J. (2004). Severity of Lung Injury in Cyclooxygenase-2-Deficient Mice Is Dependent on Reduced Prostaglandin E2 Production. Am. J. Pathol. 165: 1663-1676 [Abstract] [Full Text]
Clement, A., committee members, (2004). Task force on chronic interstitial lung disease in immunocompetent children. Eur Respir J 24: 686-697 [Abstract] [Full Text]
Nadrous, H. F., Ryu, J. H., Douglas, W. W., Decker, P. A., Olson, E. J. (2004). Impact of Angiotensin-Converting Enzyme Inhibitors and Statins on Survival in Idiopathic Pulmonary Fibrosis. Chest 126: 438-446 [Abstract] [Full Text]
Zhang, L., Keane, M. P., Zhu, L. X., Sharma, S., Rozengurt, E., Strieter, R. M., Dubinett, S. M., Huang, M. (2004). Interleukin-7 and Transforming Growth Factor-{beta} Play Counter-regulatory Roles in Protein Kinase C-{delta}-dependent Control of Fibroblast Collagen Synthesis in Pulmonary Fibrosis. J. Biol. Chem. 279: 28315-28319 [Abstract] [Full Text]
Barbarin, V., Arras, M., Misson, P., Delos, M., McGarry, B., Phan, S. H., Lison, D., Huaux, F. (2004). Characterization of the Effect of Interleukin-10 on Silica-Induced Lung Fibrosis in Mice. Am. J. Respir. Cell Mol. Bio. 31: 78-85 [Abstract] [Full Text]
Hill, A. R., Fruchter, O., Eisner, M. D., Tsoutsou, P. G., Gourgoulianis, K. I., Vourlekis, J. S., Richeldi, L., Raghu, G., King, T. E. Jr., Teirstein, A. S. (2004). Interferon Gamma-1b for Pulmonary Fibrosis. NEJM 350: 1794-1797 [Full Text]
Huaux, F., Liu, T., McGarry, B., Ullenbruch, M., Xing, Z., Phan, S. H. (2003). Eosinophils and T Lymphocytes Possess Distinct Roles in Bleomycin-Induced Lung Injury and Fibrosis. J. Immunol. 171: 5470-5481 [Abstract] [Full Text]
Kamp, D. W. (2003). Idiopathic Pulmonary Fibrosis: The Inflammation Hypothesis Revisited. Chest 124: 1187-1190 [Full Text]
Tajima, S., Oshikawa, K., Tominaga, S.-i., Sugiyama, Y. (2003). The Increase in Serum Soluble ST2 Protein Upon Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Chest 124: 1206-1214 [Abstract] [Full Text]
Hunninghake, G. W., Lynch, D. A., Galvin, J. R., Gross, B. H., Muller, N., Schwartz, D. A., King, T. E. Jr, Lynch, J. P. III, Hegele, R., Waldron, J., Colby, T. V., Hogg, J. C. (2003). Radiologic Findings Are Strongly Associated With a Pathologic Diagnosis of Usual Interstitial Pneumonia. Chest 124: 1215-1223 [Abstract] [Full Text]
Aoshiba, K., Tsuji, T., Nagai, A. (2003). Bleomycin induces cellular senescence in alveolar epithelial cells. Eur Respir J 22: 436-443 [Abstract] [Full Text]
Kaminski, N., Belperio, J. A., Bitterman, P. B., Chen, L., Chensue, S. W., Choi, A. M.K., Dacic, S., Dauber, J. H., du Bois, R. M., Enghild, J. J., Fattman, C. L., Grutters, J. C., Haegens, A., Hanford, L. E., Heintz, N., Henson, P. M., Hogaboam, C., Kagan, V. E., Keane, M. P., Kunkel, S. L., Land, S., Loyd, J. E., Lukacs, N., MacPherson, M., Manning, B., Manning, N., Martinelli, M., Moller, D. R., Morse, D., Mossman, B., Noble, P. W., Nowak, N., Oury, T. D., Pardo, A., Perez, A., Petty, T. L., Phan, S. H., Ramos-Nino, M. E., Ray, P., Rogers, R. M., Sato, H., Scapoli, L., Schaefer, L. M., Selman, M., Stern, M., Strollo, D. C., Tyurin, V. A., Valnickova, Z., Welsh, K. I., Witzmann, F. A., Yousem, S. A., Strieter, R. M. (2003). Idiopathic Pulmonary Fibrosis. Am. J. Respir. Cell Mol. Bio. 29: S1-105 [Full Text]
Card, J. W., Racz, W. J., Brien, J. F., Margolin, S. B., Massey, T. E. (2003). Differential Effects of Pirfenidone on Acute Pulmonary Injury and Ensuing Fibrosis in the Hamster Model of Amiodarone-Induced Pulmonary Toxicity. Toxicol Sci 75: 169-180 [Abstract] [Full Text]
Chung, M. P., Monick, M. M., Hamzeh, N. Y., Butler, N. S., Powers, L. S., Hunninghake, G. W. (2003). Role of Repeated Lung Injury and Genetic Background in Bleomycin-Induced Fibrosis. Am. J. Respir. Cell Mol. Bio. 29: 375-380 [Abstract] [Full Text]
Xu, Y. D., Hua, J., Mui, A., O'Connor, R., Grotendorst, G., Khalil, N. (2003). Release of biologically active TGF-{beta}1 by alveolar epithelial cells results in pulmonary fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol. 285: L527-L539 [Abstract] [Full Text]
Suzuki, H., Aoshiba, K., Yokohori, N., Nagai, A. (2003). Epidermal Growth Factor Receptor Tyrosine Kinase Inhibition Augments a Murine Model of Pulmonary Fibrosis. Cancer Res. 63: 5054-5059 [Abstract] [Full Text]
Thabut, G., Mal, H., Castier, Y., Groussard, O., Brugiere, O., Marrash-Chahla, R., Leseche, G., Fournier, M. (2003). Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J. Thorac. Cardiovasc. Surg. 126: 469-475 [Abstract] [Full Text]
Epperly, M. W., Guo, H., Gretton, J. E., Greenberger, J. S. (2003). Bone Marrow Origin of Myofibroblasts in Irradiation Pulmonary Fibrosis. Am. J. Respir. Cell Mol. Bio. 29: 213-224 [Abstract] [Full Text]
Zorzetto, M., Ferrarotti, I., Trisolini, R., Agli, L. L., Scabini, R., Novo, M., De Silvestri, A., Patelli, M., Martinetti, M., Cuccia, M., Poletti, V., Pozzi, E., Luisetti, M. (2003). Complement Receptor 1 Gene Polymorphisms Are Associated with Idiopathic Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 168: 330-334 [Abstract] [Full Text]
Endo, M., Oyadomari, S., Terasaki, Y., Takeya, M., Suga, M., Mori, M., Gotoh, T. (2003). Induction of arginase I and II in bleomycin-induced fibrosis of mouse lung. Am. J. Physiol. Lung Cell. Mol. Physiol. 285: L313-L321 [Abstract] [Full Text]
Bergeron, A., Soler, P., Kambouchner, M., Loiseau, P., Milleron, B., Valeyre, D., Hance, A.J., Tazi, A. (2003). Cytokine profiles in idiopathic pulmonary fibrosis suggest an important role for TGF-{beta} and IL-10. Eur Respir J 22: 69-76 [Abstract] [Full Text]
Strieter, R. M. (2003). To Clot or Not to Clot, That Is the Question in Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 167: 1589-1590 [Full Text]
Kinnula, V. L., Crapo, J. D. (2003). Superoxide Dismutases in the Lung and Human Lung Diseases. Am. J. Respir. Crit. Care Med. 167: 1600-1619 [Abstract] [Full Text]
McGuire, J. K., Li, Q., Parks, W. C. (2003). Matrilysin (Matrix Metalloproteinase-7) Mediates E-Cadherin Ectodomain Shedding in Injured Lung Epithelium. Am. J. Pathol. 162: 1831-1843 [Abstract] [Full Text]
Ando, M., Murakami, Y., Kojima, F., Endo, H., Kitasato, H., Hashimoto, A., Kobayashi, H., Majima, M., Inoue, M., Kondo, H., Kawai, S., Hayashi, I. (2003). Retrovirally Introduced Prostaglandin D2 Synthase Suppresses Lung Injury Induced by Bleomycin. Am. J. Respir. Cell Mol. Bio. 28: 582-591 [Abstract] [Full Text]
Chilosi, M., Poletti, V., Zamo, A., Lestani, M., Montagna, L., Piccoli, P., Pedron, S., Bertaso, M., Scarpa, A., Murer, B., Cancellieri, A., Maestro, R., Semenzato, G., Doglioni, C. (2003). Aberrant Wnt/{beta}-Catenin Pathway Activation in Idiopathic Pulmonary Fibrosis. Am. J. Pathol. 162: 1495-1502 [Abstract] [Full Text]
Selman, M. (2003). A Dark Side of Interferon-{gamma} in the Treatment of Idiopathic Pulmonary Fibrosis?. Am. J. Respir. Crit. Care Med. 167: 945-946 [Full Text]
Huaux, F., Liu, T., McGarry, B., Ullenbruch, M., Phan, S. H. (2003). Dual Roles of IL-4 in Lung Injury and Fibrosis. J. Immunol. 170: 2083-2092 [Abstract] [Full Text]
Renzoni, E. A., Walsh, D. A., Salmon, M., Wells, A. U., Sestini, P., Nicholson, A. G., Veeraraghavan, S., Bishop, A. E., Romanska, H. M., Pantelidis, P., Black, C. M., du Bois, R. M. (2003). Interstitial Vascularity in Fibrosing Alveolitis. Am. J. Respir. Crit. Care Med. 167: 438-443 [Abstract] [Full Text]
Irifune, K., Yokoyama, A., Kohno, N., Sakai, K., Hiwada, K. (2003). T-helper 1 cells induce alveolitis but do not lead to pulmonary fibrosis in mice. Eur Respir J 21: 11-18 [Abstract] [Full Text]
Kuroki, M., Noguchi, Y., Shimono, M., Tomono, K., Tashiro, T., Obata, Y., Nakayama, E., Kohno, S. (2003). Repression of Bleomycin-Induced Pneumopathy by TNF. J. Immunol. 170: 567-574 [Abstract] [Full Text]
Gong, M. N., Mark, E. J. (2002). Case 40-2002 - A 56-Year-Old Man with Rapidly Worsening Dyspnea. NEJM 347: 2149-2157 [Full Text]
Izbicki, G., Or, R., Christensen, T. G., Segel, M. J., Fine, A., Goldstein, R. H., Breuer, R. (2002). Bleomycin-induced lung fibrosis in IL-4-overexpressing and knockout mice. Am. J. Physiol. Lung Cell. Mol. Physiol. 283: L1110-L1116 [Abstract] [Full Text]
Selman, M. (2002). From Anti-inflammatory Drugs Through Antifibrotic Agents to Lung Transplantation : A Long Road of Research, Clinical Attempts, and Failures in the Treatment of Idiopathic Pulmonary Fibrosis. Chest 122: 759-761 [Full Text]
Huaux, F., Arras, M., Tomasi, D., Barbarin, V., Delos, M., Coutelier, J.-P., Vink, A., Phan, S. H., Renauld, J.-C., Lison, D. (2002). A Profibrotic Function of IL-12p40 in Experimental Pulmonary Fibrosis. J. Immunol. 169: 2653-2661 [Abstract] [Full Text]
Nicholson, A. G., Fulford, L. G., Colby, T. V., du Bois, R. M., Hansell, D. M., Wells, A. U. (2002). The Relationship between Individual Histologic Features and Disease Progression in Idiopathic Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 166: 173-177 [Abstract] [Full Text]
Tanino, Y., Makita, H., Miyamoto, K., Betsuyaku, T., Ohtsuka, Y., Nishihira, J., Nishimura, M. (2002). Role of macrophage migration inhibitory factor in bleomycin-induced lung injury and fibrosis in mice. Am. J. Physiol. Lung Cell. Mol. Physiol. 283: L156-L162 [Abstract] [Full Text]
Strieter, R. M. (2002). Inflammatory Mechanisms Are Not a Minor Component of the Pathogenesis of Idiopathic Pulmonary Fibrosis. Am. J. Respir. Crit. Care Med. 165: 1206-1207 [Full Text]

Comments and questions? Please contact us.