Future Directions in Idiopathic Pulmonary Fibrosis Research

被引：188

作者：

Blackwell, Timothy S. ^{[1
]}

Tager, Andrew M. ^{[2
]}

Borok, Zea ^{[3
]}

Moore, Bethany B. ^{[4
]}

Schwartz, David A. ^{[5
,6
]}

Anstrom, Kevin J. ^{[7
]}

Bar-Joseph, Ziv ^{[8
]}

Bitterman, Peter ^{[9
]}

Blackburn, Michael R. ^{[10
]}

Bradford, William ^{[11
]}

Brown, Kevin K. ^{[5
,6
]}

Chapman, Harold A. ^{[12
]}

Collard, Harold R. ^{[12
]}

Cosgrove, Gregory P. ^{[5
,6
]}

Deterding, Robin ^{[5
,6
]}

Doyle, Ramona ^{[13
]}

Flaherty, Kevin R. ^{[4
]}

Garcia, Christine Kim ^{[14
]}

Hagood, James S. ^{[15
]}

Henke, Craig A. ^{[9
]}

Herzog, Erica ^{[16
]}

Hogaboam, Cory M. ^{[4
]}

Horowitz, Jeffrey C. ^{[4
]}

King, Talmadge E., Jr. ^{[12
]}

Loyd, James E. ^{[1
]}

Lawson, William E. ^{[1
]}

Marsh, Clay B. ^{[17
]}

Noble, Paul W. ^{[18
]}

Noth, Imre ^{[19
]}

Sheppard, Dean ^{[12
]}

Olsson, Julie ^{[13
]}

Ortiz, Luis A. ^{[20
]}

O'Riordan, Thomas G. ^{[21
]}

Oury, Tim D. ^{[20
]}

Raghu, Ganesh ^{[22
]}

Roman, Jesse ^{[23
]}

Sime, Patricia J. ^{[24
]}

Sisson, Thomas H. ^{[4
]}

Tschumperlin, Daniel ^{[25
]}

Violette, Shelia M. ^{[26
]}

Weaver, Timothy E. ^{[27
]}

Wells, Rebecca G. ^{[28
]}

White, Eric S. ^{[4
]}

Kaminski, Naftali ^{[8
]}

Martinez, Fernando J. ^{[4
]}

Wynn, Thomas A. ^{[29
]}

Thannickal, Victor J. ^{[30
]}

Eu, Jerry P. ^{[31
]}

机构：

[1] Vanderbilt Univ, Med Ctr, Nashville, TN USA

[2] Massachusetts Gen Hosp, Boston, MA 02114 USA

[3] Univ So Calif, Los Angeles, CA USA

[4] Univ Michigan, Ann Arbor, MI 48109 USA

[5] Natl Jewish Hlth, Denver, CO USA

[6] Univ Colorado, Denver, CO 80202 USA

[7] Duke Clin Res Inst, Durham, NC USA

[8] Carnegie Mellon Univ, Pittsburgh, PA 15213 USA

[9] Univ Minnesota, Sch Med, Minneapolis, MN 55455 USA

[10] Univ Texas Houston, Med Sch Houston, Houston, TX USA

[11] InterMune Inc, Brisbane, CA USA

[12] Univ Calif San Francisco, San Francisco, CA 94143 USA

[13] Genentech Roche, San Francisco, CA USA

[14] Univ Texas SW Med Ctr Dallas, Dallas, TX 75390 USA

[15] Univ Calif San Diego, San Diego, CA 92103 USA

[16] Yale Univ, Sch Med, New Haven, CT USA

[17] Ohio State Univ, Columbus, OH 43210 USA

[18] Cedars Sinai Med Ctr, Los Angeles, CA USA

[19] Univ Chicago, Chicago, IL 60637 USA

[20] Univ Pittsburgh, Med Ctr, Pittsburgh, PA USA

[21] Gilead Sci Inc, Foster City, CA 94404 USA

[22] Univ Washington, Med Ctr, Seattle, WA 98195 USA

[23] Univ Louisville, Louisville, KY 40292 USA

[24] Univ Rochester, Sch Med, Rochester, NY USA

[25] Harvard Sch Publ Hlth, Boston, MA USA

[26] Biogen Idec Inc, Cambridge, MA USA

[27] Cincinnati Childrens Res Fdn, Cincinnati, OH USA

[28] Univ Penn, Philadelphia, PA 19104 USA

[29] NIAID, Bethesda, MD 20892 USA

[30] Univ Alabama Birmingham, Birmingham, AL USA

[31] NHLBI, Bethesda, MD 20892 USA

来源：

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE | 2014年 / 189卷 / 02期

基金：

美国国家卫生研究院;

关键词：

idiopathic pulmonary fibrosis; alveolar epithelial cells; extracellular matrix; interstitial lung disease; inflammation; MUC5B PROMOTER POLYMORPHISM; SURFACTANT PROTEIN-C; ENDOPLASMIC-RETICULUM STRESS; GROWTH-FACTOR-BETA; CIRCULATING FIBROCYTES; MESENCHYMAL TRANSITION; NEXT-GENERATION; LIVER FIBROSIS; LUNG FIBROSIS; END-POINT;

D O I：

10.1164/rccm.201306-1141WS

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

The median survival of patients with idiopathic pulmonary fibrosis (IPF) continues to be approximately 3 years from the time of diagnosis, underscoring the lack of effective medical therapies for this disease. In the United States alone, approximately 40,000 patients die of this disease annually. In November 2012, the NHLBI held a workshop aimed at coordinating research efforts and accelerating the development of IPF therapies. Basic, translational, and clinical researchers gathered with representatives from the NHLBI, patient advocacy groups, pharmaceutical companies, and the U. S. Food and Drug Administration to review the current state of IPF research and identify priority areas, opportunities for collaborations, and directions for future research. The workshop was organized into groups that were tasked with assessing and making recommendations to promote progress in one of the following six critical areas of research: (1) biology of alveolar epithelial injury and aberrant repair; (2) role of extracellular matrix; (3) preclinical modeling; (4) role of inflammation and immunity; (5) genetic, epigenetic, and environmental determinants; (6) translation of discoveries into diagnostics and therapeutics. The workshop recommendations provide a basis for directing future research and strategic planning by scientific, professional, and patient communities and the NHLBI.

引用

页码：214 / 222

页数：9

共 113 条

[31] Patients with Idiopathic Pulmonary Fibrosis with Antibodies to Heat Shock Protein 70 Have Poor Prognoses [J].