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Cardiology369 papers

Idiopathic pulmonary fibrosis

Last edited: 4/23/2026

Overview

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease characterized by irreversible fibrosis and a poor prognosis. It primarily affects older adults and is marked by worsening dyspnea and reduced lung function. 6660

Diagnosis

  • High-resolution computed tomography (HRCT) showing characteristic UIP pattern 6366
  • Exclusion of other causes of interstitial lung disease through clinical evaluation and pulmonary function tests 62
  • Autoimmune serology and lung biopsy when HRCT is inconclusive 62
  • Management

  • First-line treatments:
  • - Pirfenidone: 2403 mg/day or 2409 mg/day 1516 - Nintedanib: 150 mg bid 1516
  • Adjunctive therapies:
  • - Pulmonary rehabilitation to improve exercise tolerance and quality of life 5458 - Telerehabilitation-assisted inspiratory muscle training 19 - Consideration of palliative care early in the disease course to improve quality of life and reduce hospital visits 28

    Special Populations

  • Elderly: Increased focus on palliative care and symptom management due to higher comorbidity burden 1228
  • Comorbidities: Management of comorbidities like hypertension and gastroesophageal reflux disease (GERD) impacts overall outcomes 1340
  • Pregnancy: Limited data; treatment should be individualized with close monitoring [Not covered in abstracts]
  • Pediatrics: Not typically diagnosed; management strategies are extrapolated from adult guidelines [Not covered in abstracts]
  • Key Recommendations

  • Initiate first-line antifibrotic therapy (pirfenidone or nintedanib) in patients with confirmed IPF to slow disease progression (Evidence: Strong 1516)
  • Incorporate pulmonary rehabilitation to enhance exercise capacity and quality of life (Evidence: Moderate 5458)
  • Early integration of palliative care services to improve symptom management and patient outcomes (Evidence: Moderate 28)
  • Monitor and manage comorbidities such as hypertension and GERD, as they can influence disease progression and treatment tolerability (Evidence: Moderate 1340)
  • References

    Showing 100 most recent of 188 indexed papers.

    1 Im SI, Bae SH, Kim SJ, Kim BJ, Heo JH, Jang TW et al.. Impact of Pirfenidone on Arrhythmic and Clinical Outcomes in Patients With Idiopathic Pulmonary Fibrosis. Journal of cardiovascular electrophysiology 2026. link 2 Hu L, Liu R, Yang L, Xu M, Zhou Y, Cao Y. Bidirectional Mendelian Randomization Study: Unraveling the Link Between Idiopathic Pulmonary Fibrosis and Cardiovascular Disease. Annals of human genetics 2026. link 3 Richeldi L, Azuma A, Cottin V, Kreuter M, Maher TM, Martinez FJ et al.. Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis. The New England journal of medicine 2025. link 4 Ren F, Aliper A, Chen J, Zhao H, Rao S, Kuppe C et al.. A small-molecule TNIK inhibitor targets fibrosis in preclinical and clinical models. Nature biotechnology 2025. link 5 Xu Z, Ren F, Wang P, Cao J, Tan C, Ma D et al.. A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial. Nature medicine 2025. link 6 Golchin N, Patel A, Scheuring J, Wan V, Hofer K, Collet JP et al.. Incidence and prevalence of idiopathic pulmonary fibrosis: a systematic literature review and meta-analysis. BMC pulmonary medicine 2025. link 7 Nathan SD, Gao J, Kim HC, Chandel A, Chen H, Lu X et al.. Development and validation of a predictive 6-min walk score in patients with idiopathic pulmonary fibrosis. The European respiratory journal 2025. link 8 Walters TM, Leong MCH, Montesi SB, Ryerson CJ, Khor YH. Comorbidities in the idiopathic pulmonary fibrosis and progressive pulmonary fibrosis trial population: a systematic review and meta-analysis. European respiratory review : an official journal of the European Respiratory Society 2025. link 9 Mihart D, Crisan AF, Carunta V, Trăilă D, Tudorache E, Oancea C. Neurocognitive Impairment in Idiopathic Pulmonary Fibrosis: A Systematic Review of Current Evidence. Medical sciences (Basel, Switzerland) 2025. link 10 Liu Y, Chen X, Tang H, Jiang F, Tang Y, Zhu H et al.. Safety, Tolerability, and Pharmacokinetics of SC1011 (Sufenidone), a Novel Antifibrotic Small Molecule, in Phase 1 Studies in Healthy Subjects. Clinical and translational science 2025. link 11 Wang X. Neutral LPAR1 Antagonists for the Treatment of Idiopathic Pulmonary Fibrosis. Journal of medicinal chemistry 2025. link 12 Guidot DM, Pepin M, Hastings SN, Tighe R, Schmader K. Polypharmacy and potentially inappropriate medication (PIM) use among older veterans with idiopathic pulmonary fibrosis (IPF) - a retrospective cohort study. BMC pulmonary medicine 2025. link 13 Yin C, Guo X, Wang P, Sun W, Chen T, He Y et al.. Hypertension in idiopathic pulmonary fibrosis: evidence of immune and genetic links from clinical and genomic analyses. Respiratory research 2025. link 14 Raghu G, Richeldi L, Fernández Pérez ER, De Salvo MC, Silva RS, Song JW et al.. Pamrevlumab for Idiopathic Pulmonary Fibrosis: The ZEPHYRUS-1 Randomized Clinical Trial. JAMA 2024. link 15 Kim JS, Murray S, Yow E, Anstrom KJ, Kim HJ, Flaherty KR et al.. Comparison of Pirfenidone and Nintedanib: Post Hoc Analysis of the CleanUP-IPF Study. Chest 2024. link 16 Kou M, Jiao Y, Li Z, Wei B, Li Y, Cai Y et al.. Real-world safety and effectiveness of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis: a systematic review and meta-analysis. European journal of clinical pharmacology 2024. link 17 Ren Y, Zhang Y, Cheng Y, Qin H, Zhao H. Genetic liability of gut microbiota for idiopathic pulmonary fibrosis and lung function: a two-sample Mendelian randomization study. Frontiers in cellular and infection microbiology 2024. link 18 Wu Z, Spencer LG, Banya W, Westoby J, Tudor VA, Rivera-Ortega P et al.. Morphine for treatment of cough in idiopathic pulmonary fibrosis (PACIFY COUGH): a prospective, multicentre, randomised, double-blind, placebo-controlled, two-way crossover trial. The Lancet. Respiratory medicine 2024. link00432-0) 19 Aktan R, Tertemiz KC, Yiğit S, Özalevli S, Ozgen Alpaydin A, Uçan ES. Effects of home-based telerehabilitation-assisted inspiratory muscle training in patients with idiopathic pulmonary fibrosis: A randomized controlled trial. Respirology (Carlton, Vic.) 2024. link 20 Fang M, He B, Xiao J. Smoking and Idiopathic Pulmonary Fibrosis: A Meta-analysis. Nicotine & tobacco research : official journal of the Society for Research on Nicotine and Tobacco 2024. link 21 Dhooria S, Sharma R, Bal A, Sehgal IS, Kashyap D, Muthu V et al.. Common single nucleotide polymorphisms associated with idiopathic pulmonary fibrosis: a systematic review. European respiratory review : an official journal of the European Respiratory Society 2024. link 22 Pan T, Bai L, Zhu D, Wei Y, Zhao Q, Feng F et al.. The causal relationship between genetically predicted blood metabolites and idiopathic pulmonary fibrosis: A bidirectional two-sample Mendelian randomization study. PloS one 2024. link 23 Wang Y, Ji Z, Xu B, Li S, Xie Y. The incidence of acute exacerbation of idiopathic pulmonary fibrosis: a systematic review and meta-analysis. Scientific reports 2024. link 24 Liu X, Zhang D, Zhao F, Li S, Zhu H, Zhang X. Deciphering the role of oxidative stress genes in idiopathic pulmonary fibrosis: a multi-omics mendelian randomization approach. Genes and immunity 2024. link 25 Zhou Y, Ling T, Shi W. Current state of signaling pathways associated with the pathogenesis of idiopathic pulmonary fibrosis. Respiratory research 2024. link 26 Ziora D. May Small Airways Dysfunction (SAD) Play a Role in the Idiopathic Pulmonary Fibrosis (IPF) and May SAD Be a Therapeutic Target?. Advances in respiratory medicine 2024. link 27 Stukov Y, Rackauskas M. Bilateral orthotopic lung transplant via a clamshell thoracosternotomy from a donor with extended cold static lung preservation. Multimedia manual of cardiothoracic surgery : MMCTS 2024. link 28 Bassi I, Pastorello S, Guerrieri A, Giancotti G, Cuomo AM, Rizzelli C et al.. Early palliative care program in idiopathic pulmonary fibrosis patients favors at-home and hospice deaths, reduces unplanned medical visits, and prolongs survival: A pilot study. European journal of internal medicine 2024. link 29 Nathan SD, Chandel A, Wang Y, Xu J, Shao L, Watkins TR et al.. Derivation and validation of a noninvasive prediction tool to identify pulmonary hypertension in patients with IPF: Evolution of the model FORD. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2024. link 30 Faverio P, Maloberti A, Rebora P, Intravaia RCM, Tognola C, Toscani G et al.. Cardiovascular Structural and Functional Parameters in Idiopathic Pulmonary Fibrosis at Disease Diagnosis. High blood pressure & cardiovascular prevention : the official journal of the Italian Society of Hypertension 2024. link 31 Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S et al.. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2021 update. Full-length version. Respiratory medicine and research 2023. link 32 Maher TM, Avram C, Bortey E, Hart SP, Hirani N, Molyneux PL et al.. Nalbuphine Tablets for Cough in Patients with Idiopathic Pulmonary Fibrosis. NEJM evidence 2023. link 33 West A, Chaudhuri N, Barczyk A, Wilsher ML, Hopkins P, Glaspole I et al.. Inhaled pirfenidone solution (AP01) for IPF: a randomised, open-label, dose-response trial. Thorax 2023. link 34 Kataoka K, Nishiyama O, Ogura T, Mori Y, Kozu R, Arizono S et al.. Long-term effect of pulmonary rehabilitation in idiopathic pulmonary fibrosis: a randomised controlled trial. Thorax 2023. link 35 Nambiar A, Kellogg D, Justice J, Goros M, Gelfond J, Pascual R et al.. Senolytics dasatinib and quercetin in idiopathic pulmonary fibrosis: results of a phase I, single-blind, single-center, randomized, placebo-controlled pilot trial on feasibility and tolerability. EBioMedicine 2023. link 36 Yan H, Zhu C, Jin X, Feng G. Mendelian randomization reveals no correlations between herpesvirus infection and idiopathic pulmonary fibrosis. PloS one 2023. link 37 Cai S, Allen RJ, Wain LV, Dudbridge F. Reassessing the association of MUC5B with survival in idiopathic pulmonary fibrosis. Annals of human genetics 2023. link 38 Allen RJ, Oldham JM, Jenkins DA, Leavy OC, Guillen-Guio B, Melbourne CA et al.. Longitudinal lung function and gas transfer in individuals with idiopathic pulmonary fibrosis: a genome-wide association study. The Lancet. Respiratory medicine 2023. link00251-X) 39 Patel AJ, Walters GI, Watkins S, Rogers V, Fallouh H, Kalkat M et al.. Lung cancer resection in patients with underlying usual interstitial pneumonia: a meta-analysis. BMJ open respiratory research 2023. link 40 Reynolds CJ, Del Greco M F, Allen RJ, Flores C, Jenkins RG, Maher TM et al.. The causal relationship between gastro-oesophageal reflux disease and idiopathic pulmonary fibrosis: a bidirectional two-sample Mendelian randomisation study. The European respiratory journal 2023. link 41 Neighbors M, Li Q, Zhu SJ, Liu J, Wong WR, Jia G et al.. Bioactive lipid lysophosphatidic acid species are associated with disease progression in idiopathic pulmonary fibrosis. Journal of lipid research 2023. link 42 Chandel A, Pastre J, Valery S, King CS, Nathan SD. Derivation and validation of a simple multidimensional index incorporating exercise capacity parameters for survival prediction in idiopathic pulmonary fibrosis. Thorax 2023. link 43 Jiao XY, Song H, Liu WW, Yang JL, Wang ZW, Yang D et al.. The effect of CALIPER-derived parameters for idiopathic pulmonary fibrosis in predicting prognosis, progression, and mortality: a systematic review. European radiology 2023. link 44 Lin T, Zhou F, Mao H, Xie Z, Jin Y. Vitamin D and idiopathic pulmonary fibrosis: a two-sample mendelian randomization study. BMC pulmonary medicine 2023. link 45 Caminati A, Zompatori M, Fuccillo N, Sonaglioni A, Elia D, Cassandro R et al.. Coronary artery calcium score is a prognostic factor for mortality in idiopathic pulmonary fibrosis. Minerva medica 2023. link 46 Richeldi L, Azuma A, Cottin V, Hesslinger C, Stowasser S, Valenzuela C et al.. Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis. The New England journal of medicine 2022. link 47 Allen RJ, Stockwell A, Oldham JM, Guillen-Guio B, Schwartz DA, Maher TM et al.. Genome-wide association study across five cohorts identifies five novel loci associated with idiopathic pulmonary fibrosis. Thorax 2022. link 48 Otsubo K, Kishimoto J, Ando M, Kenmotsu H, Minegishi Y, Horinouchi H et al.. Nintedanib plus chemotherapy for nonsmall cell lung cancer with idiopathic pulmonary fibrosis: a randomised phase 3 trial. The European respiratory journal 2022. link 49 Naccache JM, Jouneau S, Didier M, Borie R, Cachanado M, Bourdin A et al.. Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial. The Lancet. Respiratory medicine 2022. link00354-4) 50 Pitre T, Mah J, Helmeczi W, Khalid MF, Cui S, Zhang M et al.. Medical treatments for idiopathic pulmonary fibrosis: a systematic review and network meta-analysis. Thorax 2022. link 51 Raghu G, Hamblin MJ, Brown AW, Golden JA, Ho LA, Wijsenbeek MS et al.. Long-term evaluation of the safety and efficacy of recombinant human pentraxin-2 (rhPTX-2) in patients with idiopathic pulmonary fibrosis (IPF): an open-label extension study. Respiratory research 2022. link 52 Pitre T, Khalid MF, Cui S, Zhang MC, Husnudinov R, Mah J et al.. Sildenafil for idiopathic pulmonary fibrosis: A systematic review and meta-analysis. Pulmonary pharmacology & therapeutics 2022. link 53 Huang SY, Cui HS, Lyu MS, Huang GR, Hou D, Yu MX. Efficacy of traditional Chinese medicine injections for treating idiopathic pulmonary fibrosis: A systematic review and network meta-analysis. PloS one 2022. link 54 Lei S, Li X, Xie Y, Li J. Clinical evidence for improving exercise tolerance and quality of life with pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A systematic review and meta-analysis. Clinical rehabilitation 2022. link 55 Wu X, Li W, Qin Z, Luo Z, Xue L, Chen Y. Comparison of 4 kinds of traditional Chinese medicine injections to assist in improving clinical indicators of patients with idiopathic pulmonary fibrosis: A systematic review and network meta-analysis. Medicine 2022. link 56 El Agha E, Wygrecka M. State of the Art in Idiopathic Pulmonary Fibrosis. Cells 2022. link 57 Chlumský J, Stehlík L, Šterclová M, Smetanová J, Zindr O. Exercise Tolerance in Patients With Idiopathic Pulmonary Fibrosis, Effect of Supplemental Oxy-Gen. Physiological research 2022. link 58 Perrotta F, Chino V, Allocca V, D'Agnano V, Bortolotto C, Bianco A et al.. Idiopathic pulmonary fibrosis and lung cancer: targeting the complexity of the pharmacological interconnection. Expert review of respiratory medicine 2022. link 59 Wu WH, Bonnet S, Shimauchi T, Toro V, Grobs Y, Romanet C et al.. Potential for inhibition of checkpoint kinases 1/2 in pulmonary fibrosis and secondary pulmonary hypertension. Thorax 2022. link 60 Lacedonia D, Correale M, Tricarico L, Scioscia G, Stornelli SR, Simone F et al.. Survival of patients with idiopathic pulmonary fibrosis and pulmonary hypertension under therapy with nintedanib or pirfenidone. Internal and emergency medicine 2022. link 61 Koteci A, Morgan AD, Portas L, Whittaker HR, Kallis C, George PM et al.. Left-sided heart failure burden and mortality in idiopathic pulmonary fibrosis: a population-based study. BMC pulmonary medicine 2022. link 62 Behr J, Günther A, Bonella F, Dinkel J, Fink L, Geiser T et al.. S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis. Respiration; international review of thoracic diseases 2021. link 63 Shen L, Zhang Y, Su Y, Weng D, Zhang F, Wu Q et al.. New pulmonary rehabilitation exercise for pulmonary fibrosis to improve the pulmonary function and quality of life of patients with idiopathic pulmonary fibrosis: a randomized control trial. Annals of palliative medicine 2021. link 64 Petnak T, Lertjitbanjong P, Thongprayoon C, Moua T. Impact of Antifibrotic Therapy on Mortality and Acute Exacerbation in Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis. Chest 2021. link 65 Hirani N, MacKinnon AC, Nicol L, Ford P, Schambye H, Pedersen A et al.. Target inhibition of galectin-3 by inhaled TD139 in patients with idiopathic pulmonary fibrosis. The European respiratory journal 2021. link 66 Nolan CM, Patel S, Barker RE, Walsh JA, Polgar O, Maddocks M et al.. Muscle stimulation in advanced idiopathic pulmonary fibrosis: a randomised placebo-controlled feasibility study. BMJ open 2021. link 67 Sakamoto S, Kataoka K, Kondoh Y, Kato M, Okamoto M, Mukae H et al.. Pirfenidone plus inhaled N-acetylcysteine for idiopathic pulmonary fibrosis: a randomised trial. The European respiratory journal 2021. link 68 Nwafor EO, Lu P, Liu Y, Peng H, Qin H, Zhang K et al.. Active Components from Traditional Herbal Medicine for the Potential Therapeutics of Idiopathic Pulmonary Fibrosis: A Systemic Review. The American journal of Chinese medicine 2021. link 69 Noth I, Cottin V, Chaudhuri N, Corte TJ, Johannson KA, Wijsenbeek M et al.. Home spirometry in patients with idiopathic pulmonary fibrosis: data from the INMARK trial. The European respiratory journal 2021. link 70 Dipla K, Boutou AK, Markopoulou A, Pitsiou G, Papadopoulos S, Chatzikosti A et al.. Exertional Desaturation in Idiopathic Pulmonary Fibrosis: The Role of Oxygen Supplementation in Modifying Cerebral-Skeletal Muscle Oxygenation and Systemic Hemodynamics. Respiration; international review of thoracic diseases 2021. link 71 Delameillieure A, Vandekerkhof S, Van Grootven B, Wuyts WA, Dobbels F. Care programs and their components for patients with idiopathic pulmonary fibrosis: a systematic review. Respiratory research 2021. link 72 Zhu S. Monocyte as a prognostic marker in patients with idiopathic pulmonary fibrosis. Respiratory research 2021. link 73 Refini RM, Bettini G, Kacerja E, Cameli P, d'Alessandro M, Bergantini L et al.. The role of the combination of echo-HRCT score as a tool to evaluate the presence of pulmonary hypertension in idiopathic pulmonary fibrosis. Internal and emergency medicine 2021. link 74 Kato S, Kitamura H, Hayakawa K, Fukui K, Tabata E, Otoshi R et al.. Coronary artery disease and heart failure in patients with idiopathic pulmonary fibrosis. Heart and vessels 2021. link 75 Yamazaki R, Nishiyama O, Yoshikawa K, Saeki S, Sano H, Iwanaga T et al.. HFpEF without elevated right ventricular systolic pressure is a favorable prognostic indicator in patients with IPF requiring hospitalization for heart failure. PloS one 2021. link 76 Piotrowski WJ, Bestry I, Białas AJ, Boros PW, Grzanka P, Jassem E et al.. Guidelines of the Polish Respiratory Society for diagnosis and treatment of idiopathic pulmonary fibrosis. Advances in respiratory medicine 2020. link 77 Werderman DS. Idiopathic Pulmonary Fibrosis. Radiologic technology 2020. link 78 Zamani A. Simple educational kit for patients diagnosed with idiopathic pulmonary fibrosis. Patient education and counseling 2020. link 79 Cruwys S, Hein P, Humphries B, Black D. Drug discovery and development in idiopathic pulmonary fibrosis: challenges and opportunities. Drug discovery today 2020. link 80 Majewski S, Lewandowska K, Martusewicz-Boros MM, Piotrowski WJ. Diagnostic and treatment standards in idiopathic pulmonary fibrosis in the era of antifibrotic drugs in Poland: A real-world practice survey. Advances in respiratory medicine 2019. link 81 LaCamera PP, Limb SL, Haselkorn T, Morgenthien EA, Stauffer JL, Wencel ML. Physician characteristics associated with treatment initiation patterns in idiopathic pulmonary fibrosis. Chronic respiratory disease 2019. link 82 D'Andrea A, Stanziola AA, Saggar R, Saggar R, Sperlongano S, Conte M et al.. Right Ventricular Functional Reserve in Early-Stage Idiopathic Pulmonary Fibrosis: An Exercise Two-Dimensional Speckle Tracking Doppler Echocardiography Study. Chest 2019. link 83 Shimomura I, Abe M, Li Y, Tsushima K, Sakao S, Tanabe N et al.. Pulmonary Hypertension Exacerbated by Nintedanib Administration for Idiopathic Pulmonary Fibrosis. Internal medicine (Tokyo, Japan) 2019. link 84 Spratt JR, Tomic R, Brown RZ, Rudser K, Loor G, Hertz M et al.. Single Versus Bilateral Lung Transplantation for Idiopathic Pulmonary Fibrosis in the Lung Allocation Score Era. The Journal of surgical research 2019. link 85 Bendstrup E, Wuyts W, Alfaro T, Chaudhuri N, Cornelissen R, Kreuter M et al.. Nintedanib in Idiopathic Pulmonary Fibrosis: Practical Management Recommendations for Potential Adverse Events. Respiration; international review of thoracic diseases 2019. link 86 Kishaba T. Acute Exacerbation of Idiopathic Pulmonary Fibrosis. Medicina (Kaunas, Lithuania) 2019. link 87 Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ et al.. Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. American journal of respiratory and critical care medicine 2018. link 88 Dos Santos CC, Gopal B, Verma S. Metformin: An Old Dog with a New Trick?. Cell metabolism 2018. link 89 Ferrara G, Luppi F, Birring SS, Cerri S, Caminati A, Sköld M et al.. Best supportive care for idiopathic pulmonary fibrosis: current gaps and future directions. European respiratory review : an official journal of the European Respiratory Society 2018. link 90 Cherrez-Ojeda I, Cottin V, Calderón JC, Delgado C, Calero E, Simanca-Racines D et al.. Management and attitudes about IPF (Idiopathic Pulmonary Fibrosis) among physicians from Latin America. BMC pulmonary medicine 2018. link 91 Salvatore M, Ishikawa G, Padilla M. Is It Idiopathic Pulmonary Fibrosis or Not?. Journal of the American Board of Family Medicine : JABFM 2018. link 92 Oda K, Yatera K, Fujino Y, Kido T, Hanaka T, Sennari K et al.. Respiratory comorbidities and risk of mortality in hospitalized patients with idiopathic pulmonary fibrosis. Respiratory investigation 2018. link 93 Zubairi ABS, Ahmad H, Hassan M, Sarwar S, Abbas A, Shahzad T et al.. Clinical characteristics and factors associated with mortality in idiopathic pulmonary fibrosis: An experience from a tertiary care center in Pakistan. The clinical respiratory journal 2018. link 94 Jacob J, Bartholmai BJ, Rajagopalan S, Karwoski R, Nair A, Walsh SLF et al.. Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema. Respirology (Carlton, Vic.) 2018. link 95 Funke-Chambour M, Azzola A, Adler D, Barazzone-Argiroffo C, Benden C, Boehler A et al.. Idiopathic Pulmonary Fibrosis in Switzerland: Diagnosis and Treatment. Respiration; international review of thoracic diseases 2017. link 96 Xaubet A, Molina-Molina M, Acosta O, Bollo E, Castillo D, Fernández-Fabrellas E et al.. Guidelines for the medical treatment of idiopathic pulmonary fibrosis. Archivos de bronconeumologia 2017. link 97 Jo HE, Troy LK, Keir G, Chambers DC, Holland A, Goh N et al.. Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia. Respirology (Carlton, Vic.) 2017. link 98 Piotrowski WJ, Martusewicz-Boros MM, Białas AJ, Lewandowska K. Idiopathic pulmonary fibrosis (IPF) - common practice in Poland before the "antifibrotic drugs era". Advances in respiratory medicine 2017. link 99 Vainshelboim B, Kramer MR, Fox BD, Izhakian S, Sagie A, Oliveira J. Supervised exercise training improves exercise cardiovascular function in idiopathic pulmonary fibrosis. European journal of physical and rehabilitation medicine 2017. link 100 Glassberg MK, Minkiewicz J, Toonkel RL, Simonet ES, Rubio GA, DiFede D et al.. Allogeneic Human Mesenchymal Stem Cells in Patients With Idiopathic Pulmonary Fibrosis via Intravenous Delivery (AETHER): A Phase I Safety Clinical Trial. Chest 2017. link

    Original source

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      Incidence and prevalence of idiopathic pulmonary fibrosis: a systematic literature review and meta-analysis.Golchin N, Patel A, Scheuring J, Wan V, Hofer K, Collet JP et al. BMC pulmonary medicine (2025)
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      Development and validation of a predictive 6-min walk score in patients with idiopathic pulmonary fibrosis.Nathan SD, Gao J, Kim HC, Chandel A, Chen H, Lu X et al. The European respiratory journal (2025)
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