Our researchers are extensively involved in increasing their understanding of Idiopathic Pulmonary Fibrosis (IPF) and the potential causes and mechanisms that lead to the disease, and developing better diagnostic methods and treatment options.

Our Researchers

Basic Research Areas

Areas of basic research include fibroblast and epithelial cell biology, microbiome in pulmonary fibrosis, and mechanisms of extracellular matrix signaling in fibrosis.

Clinical Studies

PULSE - Clinical Study to Assess the Safety and Efficacy of Pulsed Inhaled Nitric Oxide in Subjects With Pulmonary Fibrosis on Long Term Oxygen Therapy

A phase 2b, randomized, double-blind, placebo-controlled clinical study to assess the safety and efficacy of pulsed, inhaled nitric oxide (iNO) versus placebo in subjects with pulmonary fibrosis on long term oxygen therapy (Part 1 and Part 2).

  • Principal Investigator: Elizabeth Belloli, MD
  • Study Coordinator: Candace Flaherty

CleanUP IPF for the Pulmonary Trials Cooperative (CleanUp-IPF)
The purpose of this study is to compare the effect of standard care, versus standard of care plus antimicrobial therapy (co-trimoxazole or doxycycline), on clinical outcomes in patients diagnosed with idiopathic pulmonary fibrosis (IPF).

  • Principal Investigator: Elizabeth Belloli, MD
  • Study Coordinator: Candace Flaherty

Pulmonary Fibrosis Foundation Patient Registry (PFFR)
The Pulmonary Fibrosis Foundation Patient Registry will collect data on at least 2,000 patients with interstitial lung disease (ILD) at approximately 40 clinical sites in the US. The Registry is targeting enrollment of approximately 60% of the 2,000 ILD participants to have idiopathic pulmonary fibrosis (IPF). The aim of the Registry is to create a cohort of well-characterized patients with interstitial lung disease (ILD) for participation in retrospective and prospective research. 

  • Principal Investigator: Elizabeth Belloli, MD
  • Study Coordinator: Candace Flaherty

Idiopathic Pulmonary Fibrosis Prospective Outcomes Registry (IPF-PRO)
This registry will collect data on the strategies used to achieve a diagnosis of Idiopathic Pulmonary Fibrosis (IPF) and the treatment and management efforts applied throughout study follow-up, clinical outcome events and patient reported outcome data. Blood samples will be collected periodically throughout the study for use in future research efforts.

  • Principal Investigator: Kevin Flaherty, MD, MS
  • Study Coordinator: Candace Flaherty

Recent Publications

  • Wu X, Kim GH, Salisbury ML, Barber D, Bartholmai BJ, Brown KK, Conoscenti CS, De Backer J, Flaherty KR, Gruden JF, Hoffman EA, Humphries SM, Jacob J, Maher TM, Raghu G, Richeldi L, Ross BD, Schlenker-Herceg R, Sverzellati N, Wells AU, Martinez FJ, Lynch DA, Goldin J, Walsh SLF. Computed Tomographic Biomarkers in Idiopathic Pulmonary Fibrosis: The Future of Quantitative Analysis. Am J Respir Crit Care Med. 2018 Jul 9. doi: 10.1164/rccm.201803-0444PP. PMID: 29986154
  • Flaherty KR, Kolb M, Vancheri C, Tang W, Conoscenti CS, Richeldi L. Stability or Improvement in Forced Vital Capacity with Nintedanib in Patients with IPF. Eur Respir J. 2018 Jun 25. pii: 1702593. doi: 10.1183/13993003.02593-2017. PMID: 29946007
  • de Brouwer B, Drent M, van den Ouweland JMW, Wijnen PA, van Moorsel CHM, Bekers O, Grutters JC, White ES, Janssen R. Increased Circulating Desmosine and Age-Dependent Elastinolysis in Idiopathic Pulmonary Fibrosis. Respir Res. 2018 Mar 20;19(1):45. doi: 10.1186/s12931-018-0747-6. PMID: 29558926; PMCID: PMC5859529
  • Tjin G, White ES, Faiz A, Sicard D, Tschumperlin DJ, Mahar A, Kable EPW, Burgess JK. Lysyl Oxidases Regulate Fibrillar Collagen Remodelling in Idiopathic Pulmonary Fibrosis. Dis Model Mech. 2017 Nov 1;10(11):1301-1312. doi: 10.1242/dmm.030114. Erratum in: Dis Model Mech. 2017 Dec 19;10 (12 ):1545. PMID: 29125826; PMCID: PMC5719253
  • Salisbury ML, Tolle LB, Xia M, Murray S, Tayob N, Nambiar AM, Schmidt SL, Lagstein A, Myers JL, Gross BH, Kazerooni EA, Sundaram B, Chughtai AR, Martinez FJ, Flaherty KR. Possible UIP Pattern on High-Resolution Computed Tomography Is Associated with Better Survival Than Definite UIP in IPF Patients. Respir Med. 2017 Oct;131:229-235. doi: 10.1016/j.rmed.2017.08.025. Epub 2017 Sep 12. PMID: 28947036; PMCID: PMC5679475
  • Huang Y, Ma SF, Espindola MS, Vij R, Oldham JM, Huffnagle GB, Erb-Downward JR, Flaherty KR, Moore BB, White ES, Zhou T, Li J, Lussier YA, Han MK, Kaminski N, Garcia JGN, Hogaboam CM, Martinez FJ, Noth I; COMET-IPF Investigators. Microbes Are Associated with Host Innate Immune Response in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2017 Jul 15;196(2):208-219. doi: 10.1164/rccm.201607-1525OC. PMID: 28157391; PMCID: PMC5519968
  • O'Dwyer DN, Norman KC, Xia M, Huang Y, Gurczynski SJ, Ashley SL, White ESFlaherty KR, Martinez FJ, Murray S, Noth I, Arnold KB, Moore BB. Erratum: The Peripheral Blood Proteome Signature of Idiopathic Pulmonary Fibrosis Is Distinct from Normal and Is Associated with Novel Immunological Processes. Sci Rep. 2017 Jun 30;7:46860. doi: 10.1038/srep46860. PMID: 28664924; PMCID: PMC5492277
  • Salisbury ML, Lynch DA, van Beek EJ, Kazerooni EA, Guo J, Xia M, Murray S, Anstrom KJ, Yow E, Martinez FJ, Hoffman EA, Flaherty KR; IPFnet Investigators. Idiopathic Pulmonary Fibrosis: The Association Between the Adaptive Multiple Features Method and Fibrosis Outcomes. Am J Respir Crit Care Med. 2017 Apr 1;195(7):921-929. doi: 10.1164/rccm.201607-1385OC. PMID: 27767347; PMCID: PMC5387708
  • Bauer Y, White ES, de Bernard S, Cornelisse P, Leconte I, Morganti A, Roux S, Nayler O. MMP-7 Is a Predictive Biomarker of Disease Progression in Patients with Idiopathic Pulmonary Fibrosis. ERJ Open Res. 2017 Mar 22;3(1). pii: 00074-2016. doi: 10.1183/23120541.00074-2016. eCollection 2017 Jan. PMID: 28435843; PMCID: PMC5395293
  • Martinez FJ, Chisholm A, Collard HR, Flaherty KR, Myers J, Raghu G, Walsh SL, White ES, Richeldi L. The Diagnosis of Idiopathic Pulmonary Fibrosis: Current and Future Approaches. Lancet Respir Med. 2017 Jan;5(1):61-71. doi: 10.1016/S2213-2600(16)30325-3. Epub 2016 Dec 6. Review. PMID: 27932290; PMCID: PMC5524148
  • Chanda D, Kurundkar A, Rangarajan S, Locy M, Bernard K, Sharma NS, Logsdon NJ, Liu H, Crossman DK, Horowitz JC, De Langhe S, Thannickal VJ. Developmental Reprogramming in Mesenchymal Stromal Cells of Human Subjects with Idiopathic Pulmonary Fibrosis. Sci Rep. 2016 Nov 21;6:37445. doi: 10.1038/srep37445. PMID: 27869174; PMCID: PMC5116673
  • Ashley SL, Xia M, Murray S, O'Dwyer DN, Grant E, White ES, Flaherty KR, Martinez FJ, Moore BB. Six-SOMAmer Index Relating to Immune, Protease and Angiogenic Functions Predicts Progression in IPF. PLoS One. 2016 Aug 4;11(8):e0159878. doi: 10.1371/journal.pone.0159878. eCollection 2016. PMID: 27490795; PMCID: PMC4973878
  • Sisson TH, Spagnolo P. Matriptase, Protease-activated Receptor 2, and Idiopathic Pulmonary Fibrosis. Further Evidence for Signaling Pathway Redundancy in This Difficult-to-Treat Disease? Am J Respir Crit Care Med. 2016 Apr 15;193(8):816-7. doi: 10.1164/rccm.201512-2319ED. PMID: 27082530