idiopathic pulmonary fibrosis


Summary: A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.

Top Publications

  1. Zisman D, Schwarz M, Anstrom K, Collard H, Flaherty K, Hunninghake G. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363:620-8 pubmed publisher
    ..may preferentially improve blood flow to well-ventilated regions of the lung in patients with advanced idiopathic pulmonary fibrosis, which could result in improvements in gas exchange...
  2. Cottin V, Cordier J. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136:1-2 pubmed publisher
  3. Ikezoe K, Handa T, Tanizawa K, Chen Yoshikawa T, Kubo T, Aoyama A, et al. Prognostic factors and outcomes in Japanese lung transplant candidates with interstitial lung disease. PLoS ONE. 2017;12:e0183171 pubmed publisher
    ..Of the 77 candidates, 33 had idiopathic pulmonary fibrosis (IPF) and 15 had unclassifiable ILD...
  4. Nikolaou A, Kokotou M, Limnios D, Psarra A, Kokotos G. Autotaxin inhibitors: a patent review (2012-2016). Expert Opin Ther Pat. 2017;27:815-829 pubmed publisher
    ..The use of ATX inhibitors seems an attractive strategy to produce novel medicinal agents, for example anticancer agents. ..
  5. Oda K, Ishimoto H, Yamada S, Kushima H, Ishii H, Imanaga T, et al. Autopsy analyses in acute exacerbation of idiopathic pulmonary fibrosis. Respir Res. 2014;15:109 pubmed publisher
    Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is associated with high mortality. However, few studies have so far reviewed analyses of autopsy findings in patients with AE-IPF...
  6. Steffen L, Ruppert C, Hoymann H, Funke M, Ebener S, Kloth C, et al. Surfactant replacement therapy reduces acute lung injury and collapse induration-related lung remodeling in the bleomycin model. Am J Physiol Lung Cell Mol Physiol. 2017;313:L313-L327 pubmed publisher
    ..Collapse induration also occurs in acute interstitial lung disease and idiopathic pulmonary fibrosis in humans...
  7. Raghu G. Idiopathic pulmonary fibrosis: increased survival with "gastroesophageal reflux therapy": fact or fallacy?. Am J Respir Crit Care Med. 2011;184:1330-2 pubmed publisher
  8. Vaidya B, Patel R, Muth A, Gupta V. Exploitation of Novel Molecular Targets to Treat Idiopathic Pulmonary Fibrosis: A Drug Discovery Perspective. Curr Med Chem. 2017;24:2439-2458 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is the most common fibrosing lung disease and is caused by excessive lung scarring...
  9. Eickelberg O, Laurent G. The quest for the initial lesion in idiopathic pulmonary fibrosis: gene expression differences in IPF fibroblasts. Am J Respir Cell Mol Biol. 2010;42:1-2 pubmed publisher

More Information


  1. Mathai S, Schwartz D. Taking the "I" out of IPF. Eur Respir J. 2015;45:1539-41 pubmed publisher
  2. Ley B, Collard H. Risk prediction in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2012;185:6-7 pubmed publisher
  3. Loeh B, Drakopanagiotakis F, Bandelli G, von der Beck D, Tello S, Cordani E, et al. Intraindividual response to treatment with pirfenidone in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2015;191:110-3 pubmed publisher
  4. Bournazos S, Fahim A, Hart S. Identification of fibrocytes in peripheral blood. Am J Respir Crit Care Med. 2009;180:1279; author reply 1279 pubmed
  5. Saito S, Zhuang Y, Shan B, Danchuk S, Luo F, Korfei M, et al. Tubastatin ameliorates pulmonary fibrosis by targeting the TGFβ-PI3K-Akt pathway. PLoS ONE. 2017;12:e0186615 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal disease...
  6. Ryerson C, O Connor D, Dunne J, Schooley F, Hague C, Murphy D, et al. Predicting Mortality in Systemic Sclerosis-Associated Interstitial Lung Disease Using Risk Prediction Models Derived From Idiopathic Pulmonary Fibrosis. Chest. 2015;148:1268-1275 pubmed publisher
    Mortality risk prediction tools have been developed in idiopathic pulmonary fibrosis, however, it is unknown whether these models accurately estimate mortality in systemic sclerosis-associated interstitial lung disease (SSc-ILD)...
  7. Richeldi L. Efficacy endpoints for idiopathic pulmonary fibrosis trials. Lancet Respir Med. 2015;3:335-7 pubmed publisher
  8. Wang K, Ju Q, Cao J, Tang W, Zhang J. Impact of serum SP-A and SP-D levels on comparison and prognosis of idiopathic pulmonary fibrosis: A systematic review and meta-analysis. Medicine (Baltimore). 2017;96:e7083 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) has a poor prognosis in general; however, it is heterogeneous to detect relative biomarkers for predicting the disease progression...
  9. Karimi Shah B, Chowdhury B. Forced vital capacity in idiopathic pulmonary fibrosis--FDA review of pirfenidone and nintedanib. N Engl J Med. 2015;372:1189-91 pubmed publisher
  10. Corte T, Goh N, Glaspole I, Zappala C, Hopkins P, Wilsher M. Idiopathic pulmonary fibrosis: is all-cause mortality a practical and realistic end-point for clinical trials?. Thorax. 2013;68:491-2 pubmed publisher
  11. Glassberg M, Hare J, Toonkel R, Matthay M. Reply: idiopathic pulmonary fibrosis: a degenerative disease requiring a regenerative approach. Am J Respir Crit Care Med. 2013;188:253-4 pubmed publisher
  12. Gansner J, Rosas I, Ebert B. Pulmonary fibrosis, bone marrow failure, and telomerase mutation. N Engl J Med. 2012;366:1551-3 pubmed publisher
  13. Guenther A. The European IPF Network: towards better care for a dreadful disease. Eur Respir J. 2011;37:747-8 pubmed publisher
  14. Scotton C, Chambers R. Bleomycin revisited: towards a more representative model of IPF?. Am J Physiol Lung Cell Mol Physiol. 2010;299:L439-41 pubmed publisher
  15. Lee S, Kim D, Kim Y, Chung M, Uh S, Park C, et al. Association between occupational dust exposure and prognosis of idiopathic pulmonary fibrosis: a Korean national survey. Chest. 2015;147:465-474 pubmed publisher
    Previous studies have investigated the relationship between occupational and environmental agents and idiopathic pulmonary fibrosis (IPF)...
  16. Yamaguchi R, Sakamoto A, Yamamoto T, Ishimaru Y, Narahara S, Sugiuchi H, et al. Surfactant Protein D Inhibits Interleukin-12p40 Production by Macrophages Through the SIRPα/ROCK/ERK Signaling Pathway. Am J Med Sci. 2017;353:559-567 pubmed publisher
    Interleukin (IL)-12 has a pivotal profibrotic role in the development of idiopathic pulmonary fibrosis (IPF). Medical research trials based on IPF registry databases have actively recruited patients...
  17. Togami K, Yamaguchi K, Chono S, Tada H. Evaluation of permeability alteration and epithelial-mesenchymal transition induced by transforming growth factor-β1 in A549, NCI-H441, and Calu-3 cells: Development of an in vitro model of respiratory epithelial cells in idiopathic pulmonary fibrosi. J Pharmacol Toxicol Methods. 2017;86:19-27 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a lethal lung disease, which is accompanied by changes in lung structure...
  18. Hunninghake G. A new hope for idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2142-3 pubmed publisher
  19. Wuyts W, Thomeer M, Demedts M, Verleden G. New idiopathic pulmonary fibrosis guidelines: some unresolved questions. Am J Respir Crit Care Med. 2012;185:588; author reply 588-9 pubmed
  20. Yan Z, Kui Z, Ping Z. Reviews and prospectives of signaling pathway analysis in idiopathic pulmonary fibrosis. Autoimmun Rev. 2014;13:1020-5 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing disease with disappointing survival rate, and uneffective therapeutic progress has been made in the last few years, forcing the urgent need to improve research to this ..
  21. Brown A, Fischer C, Shlobin O, Buhr R, Ahmad S, Weir N, et al. Outcomes after hospitalization in idiopathic pulmonary fibrosis: a cohort study. Chest. 2015;147:173-179 pubmed publisher
    The outcomes of patients with idiopathic pulmonary fibrosis (IPF) who undergo hospitalization have not been well characterized...
  22. Zhang Y, Noth I, Garcia J, Kaminski N. A variant in the promoter of MUC5B and idiopathic pulmonary fibrosis. N Engl J Med. 2011;364:1576-7 pubmed publisher
  23. Martinez F, Flaherty K. Comprehensive and Individualized Patient Care in Idiopathic Pulmonary Fibrosis: Refining Approaches to Diagnosis, Prognosis, and Treatment. Chest. 2017;151:1173-1174 pubmed publisher
    As seen in this CME online activity (available at, idiopathic pulmonary fibrosis (IPF) is a specific form of chronic progressive fibrotic lung disease of unknown cause...
  24. Richeldi L. Idiopathic pulmonary fibrosis: current challenges and future perspectives. Eur Respir Rev. 2013;22:103-5 pubmed publisher
  25. Kaarteenaho R. The current position of surgical lung biopsy in the diagnosis of idiopathic pulmonary fibrosis. Respir Res. 2013;14:43 pubmed publisher
    ..statement defines usual interstitial pneumonia (UIP) which is a histological and radiological form of idiopathic pulmonary fibrosis (IPF) more precisely than previously...
  26. Ackermann M, Kim Y, Wagner W, Schuppan D, Valenzuela C, Mentzer S, et al. Effects of nintedanib on the microvascular architecture in a lung fibrosis model. Angiogenesis. 2017;20:359-372 pubmed publisher
    Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis, has anti-fibrotic, anti-inflammatory, and anti-angiogenic activity...
  27. Peljto A, Selman M, Kim D, Murphy E, Tucker L, Pardo A, et al. The MUC5B promoter polymorphism is associated with idiopathic pulmonary fibrosis in a Mexican cohort but is rare among Asian ancestries. Chest. 2015;147:460-464 pubmed publisher
    Polymorphisms in the MUC5B promoter, TOLLIP, and nine additional genetic loci have been associated with idiopathic pulmonary fibrosis (IPF) within non-Hispanic white populations...
  28. Collum S, Amione Guerra J, Cruz Solbes A, DiFrancesco A, Hernandez A, Hanmandlu A, et al. Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis: Current and Future Perspectives. Can Respir J. 2017;2017:1430350 pubmed publisher
    ..present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO)...
  29. Sathiyamoorthy G, Sehgal S, Ashton R. Pirfenidone and Nintedanib for Treatment of Idiopathic Pulmonary Fibrosis. South Med J. 2017;110:393-398 pubmed publisher
    b>Idiopathic pulmonary fibrosis is one of the most common entities of the family of disorders known as the interstitial lung diseases. It is a chronic, progressive, and often-fatal disease with a median survival time of 3 to 5 years...
  30. Tomassetti S, Gurioli C, Ryu J, Decker P, Ravaglia C, Tantalocco P, et al. The impact of lung cancer on survival of idiopathic pulmonary fibrosis. Chest. 2015;147:157-164 pubmed publisher
    Lung cancer (LC) is frequently associated with idiopathic pulmonary fibrosis (IPF). Despite this well-known association, the outcome of LC in patients with IPF is unclear...
  31. Huie T, Moss M, Frankel S. What can biomarkers tell us about the pathogenesis of acute exacerbations of idiopathic pulmonary fibrosis?. Am J Physiol Lung Cell Mol Physiol. 2010;299:L1-2 pubmed publisher
  32. Kim E, Choi S, Lee J, Park Y, Lee C, Yim J, et al. Validation of the GAP score in Korean patients with idiopathic pulmonary fibrosis. Chest. 2015;147:430-437 pubmed publisher the GAP (gender, age, and physiologic variables) model matches the observed mortality from idiopathic pulmonary fibrosis (IPF) in non-Western populations...
  33. Lindell K, Liang Z, Hoffman L, Rosenzweig M, Saul M, Pilewski J, et al. Palliative care and location of death in decedents with idiopathic pulmonary fibrosis. Chest. 2015;147:423-429 pubmed publisher
    Palliative care, integrated early, may reduce symptom burden in patients with idiopathic pulmonary fibrosis (IPF). However, limited information exists on timing and clinical practice...
  34. Cottin V. The safety and tolerability of nintedanib in the treatment of idiopathic pulmonary fibrosis. Expert Opin Drug Saf. 2017;16:857-865 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease that primarily affects older individuals. Nintedanib, a tyrosine kinase inhibitor, has been approved for the treatment of IPF in several countries...
  35. Martinez F, de Andrade J, Anstrom K, King T, Raghu G. Randomized trial of acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370:2093-101 pubmed publisher
    Acetylcysteine has been suggested as a beneficial treatment for idiopathic pulmonary fibrosis, although data from placebo-controlled studies are lacking...
  36. Strand M, Sprunger D, Cosgrove G, Fernandez Perez E, Frankel S, Huie T, et al. Pulmonary function and survival in idiopathic vs secondary usual interstitial pneumonia. Chest. 2014;146:775-785 pubmed publisher
    ..CTD), but it is most commonly found in the absence of a known cause, in the clinical context of idiopathic pulmonary fibrosis (IPF)...
  37. Ryerson C, Vittinghoff E, Ley B, Lee J, Mooney J, Jones K, et al. Predicting survival across chronic interstitial lung disease: the ILD-GAP model. Chest. 2014;145:723-728 pubmed publisher
    ..model based on sex, age, and lung physiology, that was previously validated in patients with idiopathic pulmonary fibrosis. Patients with idiopathic pulmonary fibrosis (n=307), chronic hypersensitivity pneumonitis (n=206), ..
  38. Raghu G. Idiopathic pulmonary fibrosis: guidelines for diagnosis and clinical management have advanced from consensus-based in 2000 to evidence-based in 2011. Eur Respir J. 2011;37:743-6 pubmed publisher
  39. Desroy N, Housseman C, Bock X, Joncour A, Bienvenu N, Cherel L, et al. Discovery of 2-[[2-Ethyl-6-[4-[2-(3-hydroxyazetidin-1-yl)-2-oxoethyl]piperazin-1-yl]-8-methylimidazo[1,2-a]pyridin-3-yl]methylamino]-4-(4-fluorophenyl)thiazole-5-carbonitrile (GLPG1690), a First-in-Class Autotaxin Inhibitor Undergoing Clinical Evalua. J Med Chem. 2017;60:3580-3590 pubmed publisher
    ..Compound 11 is currently being evaluated in an exploratory phase 2a study in idiopathic pulmonary fibrosis patients.
  40. Yamada Y, Yamada G, Otsuka M, Nishikiori H, Ikeda K, Umeda Y, et al. Volatile Organic Compounds in Exhaled Breath of Idiopathic Pulmonary Fibrosis for Discrimination from Healthy Subjects. Lung. 2017;195:247-254 pubmed publisher find the characteristic volatile organic compounds (VOCs) in the exhaled breath of patients with idiopathic pulmonary fibrosis (IPF) for discrimination from healthy subjects...
  41. Kim D. Acute exacerbations in patients with idiopathic pulmonary fibrosis. Respir Res. 2013;14:86 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing interstitial lung disease that primarily affects older adults. Median survival after diagnosis is 2-3 years...
  42. Behr J. A small change in FVC but a big change for IPF: defining the minimal clinically important difference. Am J Respir Crit Care Med. 2011;184:1329-30 pubmed publisher
  43. Raghu G, Thickett D. Pirfenidone for IPF: pro/con debate; the 'con' viewpoint. Thorax. 2013;68:605-8 pubmed publisher
  44. Guiot J, Corhay J, Louis R. [Idiopathic pulmonary fibrosis]. Rev Med Liege. 2014;69:605-10 pubmed
    b>Idiopathic pulmonary fibrosis (IPF) is one of the multiple pathologies included in the large family of diffuse interstitial parenchymal lung diseases (IPD)...
  45. Jaffar J, Unger S, Corte T, Keller M, Wolters P, Richeldi L, et al. Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis. Chest. 2014;146:1055-1063 pubmed publisher
    The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown...
  46. Wells A, Behr J, Costabel U, Cottin V, Poletti V, Richeldi L. Hot of the breath: mortality as a primary end-point in IPF treatment trials: the best is the enemy of the good. Thorax. 2012;67:938-40 pubmed publisher
  47. Buzan M, Wetscherek A, Heussel C, Kreuter M, Herth F, Warth A, et al. Texture analysis using proton density and T2 relaxation in patients with histological usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP). PLoS ONE. 2017;12:e0177689 pubmed publisher
    ..05. T2 relaxation times and PD values may provide helpful quantitative information for differentiating NSIP from UIP pattern. These parameters have the potential to differentiate active-inflammatory and stable-fibrotic lesions in NSIP. ..
  48. Fukihara J, Taniguchi H, Ando M, Kondoh Y, Kimura T, Kataoka K, et al. Hemosiderin-laden macrophages are an independent factor correlated with pulmonary vascular resistance in idiopathic pulmonary fibrosis: a case control study. BMC Pulm Med. 2017;17:30 pubmed publisher
    Increases in hemosiderin-laden macrophages (HLM) are reported to be observed in idiopathic pulmonary fibrosis (IPF)...
  49. Kataoka K, Taniguchi H, Kondoh Y, Nishiyama O, Kimura T, Matsuda T, et al. Recombinant Human Thrombomodulin in Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Chest. 2015;148:436-443 pubmed publisher
    Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) presents as episodes of acute respiratory worsening closely associated with endothelial damage and disordered coagulopathy...
  50. Mora A, Rojas M, Pardo A, Selman M. Emerging therapies for idiopathic pulmonary fibrosis, a progressive age-related disease. Nat Rev Drug Discov. 2017;16:755-772 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a fatal age-associated disease that is characterized by progressive and irreversible scarring of the lung...
  51. Ryerson C, Corte T, Collard H, Richeldi L. A global registry for idiopathic pulmonary fibrosis: the time is now. Eur Respir J. 2014;44:273-6 pubmed publisher
  52. Bradford W, COHEN A, Leff J. Selection of clinically meaningful primary endpoints in phase 3 clinical trials in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2013;187:1269-70 pubmed publisher
  53. Ghatak S, Hascall V, Markwald R, Feghali Bostwick C, Artlett C, Gooz M, et al. Transforming growth factor ?1 (TGF?1)-induced CD44V6-NOX4 signaling in pathogenesis of idiopathic pulmonary fibrosis. J Biol Chem. 2017;292:10490-10519 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a progressive clinical syndrome of fatal outcome...
  54. Makdisi G, Haddad T, Makdisi P, Bittner H. Tuberculous Empyema Post Bilateral Lung Transplant. Ann Thorac Surg. 2017;103:e419-e421 pubmed publisher
    ..The recipient underwent video-assisted thoracic surgery for diagnosis and decortication. Both the recipient and donor lacked a history of tuberculosis or tuberculosis exposure. ..
  55. Xia H, Gilbertsen A, Herrera J, Racila E, Smith K, Peterson M, et al. Calcium-binding protein S100A4 confers mesenchymal progenitor cell fibrogenicity in idiopathic pulmonary fibrosis. J Clin Invest. 2017;127:2586-2597 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a prevalence of 1 million persons worldwide. The fibrosis spreads from affected alveoli into contiguous alveoli and leads to death by asphyxiation...
  56. Humphries S, Yagihashi K, Huckleberry J, Rho B, Schroeder J, Strand M, et al. Idiopathic Pulmonary Fibrosis: Data-driven Textural Analysis of Extent of Fibrosis at Baseline and 15-Month Follow-up. Radiology. 2017;285:270-278 pubmed publisher
    ..of thin-section computed tomography (CT) images at baseline and at 15-month follow-up in subjects with idiopathic pulmonary fibrosis (IPF)...
  57. Ley B, Swigris J, Day B, Stauffer J, Raimundo K, Chou W, et al. Pirfenidone Reduces Respiratory-related Hospitalizations in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2017;196:756-761 pubmed publisher
    Respiratory-related hospitalizations of patients with idiopathic pulmonary fibrosis (IPF) are more frequent than those for acute IPF exacerbations and are associated with poor outcomes.
  58. ROBBINS L. Idiopathic pulmonary fibrosis; roentgenologic findings. Radiology. 1948;51:459-67 pubmed
  59. Tzilas V, Bouros D. Inherent weaknesses of the current ICD coding system regarding idiopathic pulmonary fibrosis. Eur Respir J. 2015;45:1194-6 pubmed publisher
  60. Papiris S, Manali E, Kolilekas L, Triantafillidou C, Tsangaris I, Kagouridis K. Steroids in idiopathic pulmonary fibrosis acute exacerbation: defenders or killers?. Am J Respir Crit Care Med. 2012;185:587-8 pubmed
  61. Morell F. Idiopathic pulmonary fibrosis: importance of accurate diagnosis and treatment. Arch Bronconeumol. 2013;49:319-20 pubmed publisher
  62. Costabel U. Idiopathic pulmonary fibrosis: recent milestones in disease management. Eur Respir Rev. 2012;21:140 pubmed publisher
  63. Wyman A, Noor Z, Fishelevich R, Lockatell V, Shah N, Todd N, et al. Sirtuin 7 is decreased in pulmonary fibrosis and regulates the fibrotic phenotype of lung fibroblasts. Am J Physiol Lung Cell Mol Physiol. 2017;312:L945-L958 pubmed publisher
    ..the mRNA and protein levels of all seven known sirtuins in primary lung fibroblasts from patients with idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in comparison with lung ..
  64. Raghu G, Rochwerg B, Zhang Y, García C, Azuma A, Behr J, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192:e3-19 pubmed publisher
    ..Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association guideline on idiopathic pulmonary fibrosis treatment...
  65. Collard H, Brown K, Martinez F, Raghu G, Roberts R, Anstrom K. Study design implications of death and hospitalization as end points in idiopathic pulmonary fibrosis. Chest. 2014;146:1256-1262 pubmed publisher
    The feasibility of an interventional clinical trial in idiopathic pulmonary fibrosis (IPF) using death and hospitalization as primary end points is an area of uncertainty...
  66. Richeldi L. Treatments for idiopathic pulmonary fibrosis. N Engl J Med. 2014;371:783 pubmed publisher
  67. Onorato J, Shipkova P, Minnich A, Aubry A, Easter J, Tymiak A. Challenges in accurate quantitation of lysophosphatidic acids in human biofluids. J Lipid Res. 2014;55:1784-96 pubmed publisher
    ..was qualified using a stable isotope-labeled LPA as a surrogate calibrant and used to determine LPA levels in human BALF and plasma from a Phase 0 clinical study comparing idiopathic pulmonary fibrosis patients to healthy controls.
  68. O Connell O, Egan J. The burden of disease and the need for a simple staging system in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2014;189:765-7 pubmed publisher
  69. Rose D, Montgomery A. Idiopathic pulmonary fibrosis: clinically meaningful primary endpoints in phase 3 clinical trials. Am J Respir Crit Care Med. 2013;187:1269 pubmed publisher
  70. Shetty S, Tiwari N, Marudamuthu A, Puthusseri B, Bhandary Y, Fu J, et al. p53 and miR-34a Feedback Promotes Lung Epithelial Injury and Pulmonary Fibrosis. Am J Pathol. 2017;187:1016-1034 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease. The pathogenesis of interstitial lung diseases, including its most common form, IPF, remains poorly understood...
  71. Guiot J, Henket M, Corhay J, Moermans C, Louis R. Sputum biomarkers in IPF: Evidence for raised gene expression and protein level of IGFBP-2, IL-8 and MMP-7. PLoS ONE. 2017;12:e0171344 pubmed publisher
    b>Idiopathic pulmonary fibrosis (IPF) is a rare lung disease of unknown origin leading rapidly to death...
  72. Wilson K, Raghu G. The 2015 guidelines for idiopathic pulmonary fibrosis: an important chapter in the evolution of the management of patients with IPF. Eur Respir J. 2015;46:883-6 pubmed publisher