{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T21:21:03Z","timestamp":1792704063706,"version":"4.5.0"},"reference-count":145,"publisher":"Oxford University Press (OUP)","issue":"8","license":[{"start":{"date-parts":[[2014,10,1]],"date-time":"2014-10-01T00:00:00Z","timestamp":1412121600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,10,15]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Idiopathic pulmonary fibrosis (IPF) is a devastating disease, with a median survival as short as 3 years from the time of diagnosis and no pharmacological therapies yet approved by the U.S. Food and Drug Administration. To address the great unmet need for effective IPF therapy, a number of new drugs have recently been, or are now being, evaluated in clinical trials. The rationales for most of these therapeutic candidates are based on the current paradigm of IPF pathogenesis, in which recurrent injury to the alveolar epithelium is believed to drive aberrant wound healing responses, resulting in fibrosis rather than repair. Here we discuss drugs in recently completed or currently ongoing phase II and III IPF clinical trials in the context of their putative mechanisms of action and the aberrant repair processes they are believed to target: innate immune activation and polarization, fibroblast accumulation and myofibroblast differentiation, or extracellular matrix deposition and stiffening. Placed in this context, the positive results of recently completed trials of pirfenidone and nintedanib, and results that will come from ongoing trials of other agents, should provide valuable insights into the still-enigmatic pathogenesis of this disease, in addition to providing benefits to patients with IPF.<\/jats:p>","DOI":"10.1164\/rccm.201403-0509pp","type":"journal-article","created":{"date-parts":[[2014,10,15]],"date-time":"2014-10-15T12:49:45Z","timestamp":1413377385000},"page":"867-878","source":"Crossref","is-referenced-by-count":214,"title":["New Therapeutic Targets in Idiopathic Pulmonary Fibrosis. Aiming to Rein in Runaway Wound-Healing Responses"],"prefix":"10.1093","volume":"190","author":[{"given":"Neil","family":"Ahluwalia","sequence":"first","affiliation":[{"name":"Pulmonary and Critical Care Unit and Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barry S.","family":"Shea","sequence":"additional","affiliation":[{"name":"Pulmonary and Critical Care Unit and Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M.","family":"Tager","sequence":"additional","affiliation":[{"name":"Pulmonary and Critical Care Unit and Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,10,1]]},"reference":[{"key":"2026032100300574900_bib1","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1164\/rccm.2009-040GL","article-title":"An official ATS\/ERS\/JRS\/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management","volume":"183","author":"Raghu","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100300574900_bib2","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1164\/rccm.200602-163OC","article-title":"Incidence and prevalence of idiopathic pulmonary fibrosis","volume":"174","author":"Raghu","year":"2006","journal-title":"Am J Respir Crit Care Med"},{"key":"2026032100300574900_bib3","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1136\/thx.2004.035220","article-title":"Health-related quality of life in patients with idiopathic pulmonary fibrosis: a systematic review","volume":"60","author":"Swigris","year":"2005","journal-title":"Thorax"},{"key":"2026032100300574900_bib4","doi-asserted-by":"crossref","first-page":"2071","DOI":"10.1056\/NEJMoa1402584","article-title":"Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis","volume":"370","author":"Richeldi","year":"2014","journal-title":"N Engl J Med"},{"key":"2026032100300574900_bib5","doi-asserted-by":"crossref","first-page":"2083","DOI":"10.1056\/NEJMoa1402582","article-title":"A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis","volume":"370","author":"King","year":"2014","journal-title":"N Engl J Med"},{"key":"2026032100300574900_bib6","doi-asserted-by":"crossref","first-page":"136","DOI":"10.7326\/0003-4819-134-2-200101160-00015","article-title":"Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy","volume":"134","author":"Selman","year":"2001","journal-title":"Ann Intern Med"},{"key":"2026032100300574900_bib7","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1164\/ajrccm\/140.6.1693","article-title":"An immunohistochemical study of architectural remodeling and connective tissue synthesis in pulmonary fibrosis","volume":"140","author":"Kuhn","year":"1989","journal-title":"Am Rev Respir Dis"},{"key":"2026032100300574900_bib8","first-page":"443","article-title":"Intraluminal fibrosis in interstitial lung disorders","volume":"122","author":"Basset","year":"1986","journal-title":"Am J Pathol"},{"key":"2026032100300574900_bib9","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1165\/rcmb.2014-0089PS","article-title":"Alveolar epithelium: beyond the barrier","volume":"50","author":"Borok","year":"2014","journal-title":"Am J Respir Cell Mol Biol"},{"key":"2026032100300574900_bib10","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1164\/rccm.201306-1141WS","article-title":"Future directions in idiopathic pulmonary fibrosis research. 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