Evaluating the consistency with guideline recommendations for diagnosis and management of idiopathic pulmonary fibrosis in non-academic settings
Main Article Content
Keywords
idiopathic pulmonary fibrosis, diagnosis, adherence to guidelines
Abstract
Background: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with elevated mortality. Delay in diagnosis lead to worse outcomes. Guidelines developed at academic medical centers are difficult to replicate in the community.
Objectives: Our primary objective was to ascertain consistency with the 2011 IPF guidelines. Our secondary objective was to conduct an interdisciplinary review to ascertain whether the evidence supported the original diagnosis of IPF or not.
Methods: We asked permission from pulmonologists to review records of patients diagnosed with IPF after 2011. We collected physician demographics and training data; patient demographics, clinical and diagnostic/management data. The clinical data and available images were reviewed by the interdisciplinary review panel.
Results: 26 practicing pulmonologists located in the Southeast of the United States consented to participate. Mean age was 48, 70% were male and all had current certification. We reviewed data from 96 patients. The mean age was 71.4 and most were male. Only 23% had the recommended screening for a connective tissue disease and 42.6% were screened for exercise-induced hypoxemia. Among patients with available images for review (n=66), only 50% had a high-resolution CT scan. 22% of patients underwent a surgical biopsy and in only 33% of the cases three lobes were sampled. No patient had documentation that a multidisciplinary discussion occurred. In 20% of the cases with available images, the evidence supported an alternative diagnosis. 56% of eligible candidates were ever started on anti-fibrotics.
Conclusions: Our findings suggest that consistency with the IPF guidelines is low in non-academic settings.
References
2. Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med. 2011;184(7):842-7. Epub 2011/07/02. doi: 10.1164/rccm.201104-0668OC. PubMed PMID: 21719755; PubMed Central PMCID: PMCPMC3208648.
3. King TE, Jr., Bradford WZ, Castro-Bernardini S, Fagan EA, Glaspole I, Glassberg MK, et al. A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2083-92. Epub 2014/05/20. doi: 10.1056/NEJMoa1402582. PubMed PMID: 24836312.
4. Richeldi L, du Bois RM, Raghu G, Azuma A, Brown KK, Costabel U, et al. Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med. 2014;370(22):2071-82. Epub 2014/05/20. doi: 10.1056/NEJMoa1402584. PubMed PMID: 24836310.
5. Ahluwalia N, Shea BS, Tager AM. New therapeutic targets in idiopathic pulmonary fibrosis. Aiming to rein in runaway wound-healing responses. Am J Respir Crit Care Med. 2014;190(8):867-78. Epub 2014/08/05. doi: 10.1164/rccm.201403-0509PP. PubMed PMID: 25090037; PubMed Central PMCID: PMCPMC4299574.
6. Blackwell TS, Tager AM, Borok Z, Moore BB, Schwartz DA, Anstrom KJ, et al. Future directions in idiopathic pulmonary fibrosis research. An NHLBI workshop report. Am J Respir Crit Care Med. 2014;189(2):214-22. Epub 2013/10/29. doi: 10.1164/rccm.201306-1141WS. PubMed PMID: 24160862; PubMed Central PMCID: PMCPMC3983890.
7. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. Epub 2011/04/08. doi: 10.1164/rccm.2009-040GL. PubMed PMID: 21471066; PubMed Central PMCID: PMCPMC5450933.
8. Raghu G, Rochwerg B, Zhang Y, Garcia CA, 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(2):e3-19. Epub 2015/07/16. doi: 10.1164/rccm.201506-1063ST. PubMed PMID: 26177183.
9. 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. Am J Respir Crit Care Med. 2018;198(5):e44-e68. Epub 2018/09/01. doi: 10.1164/rccm.201807-1255ST. PubMed PMID: 30168753.
10. Flaherty KR, Andrei AC, King TE, Jr., Raghu G, Colby TV, Wells A, et al. Idiopathic interstitial pneumonia: do community and academic physicians agree on diagnosis? Am J Respir Crit Care Med. 2007;175(10):1054-60. Epub 2007/01/27. doi: 10.1164/rccm.200606-833OC. PubMed PMID: 17255566; PubMed Central PMCID: PMCPMC1899268.
11. Radiology ACoRaSoT. ACR-STR Practice Parameter For The Performance of High-Resolution Computed Tomography (HRCT) of the Lungs in Adults. https://www.acr.org/-/media/ACR/Files/Practice-Parameters/HRCT-Lungs.pdf20156.
12. Lynch DA, Sverzellati N, Travis WD, Brown KK, Colby TV, Galvin JR, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138-53. Epub 2017/11/21. doi: 10.1016/S2213-2600(17)30433-2. PubMed PMID: 29154106.
13. Bland JM, Altman DG. The logrank test. BMJ. 2004;328(7447):1073. Epub 2004/05/01. doi: 10.1136/bmj.328.7447.1073. PubMed PMID: 15117797; PubMed Central PMCID: PMCPMC403858.
14. Snyder L, Neely ML, Hellkamp AS, O'Brien E, de Andrade J, Conoscenti CS, et al. Predictors of death or lung transplant after a diagnosis of idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry. Respir Res. 2019;20(1):105. Epub 2019/05/31. doi: 10.1186/s12931-019-1043-9. PubMed PMID: 31142314; PubMed Central PMCID: PMCPMC6542049.
15. Kreuter M, Walscher J, Behr J. Antifibrotic drugs as treatment of nonidiopathic pulmonary fibrosis interstitial pneumonias: the time is now (?). Curr Opin Pulm Med. 2017;23(5):418-25. Epub 2017/06/18. doi: 10.1097/MCP.0000000000000408. PubMed PMID: 28622199.
16. Walsh SLF, Wells AU, Desai SR, Poletti V, Piciucchi S, Dubini A, et al. Multicentre evaluation of multidisciplinary team meeting agreement on diagnosis in diffuse parenchymal lung disease: a case-cohort study. Lancet Respir Med. 2016;4(7):557-65. Epub 2016/05/18. doi: 10.1016/S2213-2600(16)30033-9. PubMed PMID: 27180021.
17. Peikert T, Daniels CE, Beebe TJ, Meyer KC, Ryu JH, Interstitial Lung Diseases Network of the American College of Chest P. Assessment of current practice in the diagnosis and therapy of idiopathic pulmonary fibrosis. Respir Med. 2008;102(9):1342-8. Epub 2008/07/16. doi: 10.1016/j.rmed.2008.03.018. PubMed PMID: 18621518.
18. Behr J, Kreuter M, Hoeper MM, Wirtz H, Klotsche J, Koschel D, et al. Management of patients with idiopathic pulmonary fibrosis in clinical practice: the INSIGHTS-IPF registry. Eur Respir J. 2015;46(1):186-96. Epub 2015/04/04. doi: 10.1183/09031936.00217614. PubMed PMID: 25837040; PubMed Central PMCID: PMCPMC4486374.
19. Cottin V, Cadranel J, Crestani B, Dalphin JC, Delaval P, Israel-Biet D, et al. Management of idiopathic pulmonary fibrosis in France: a survey of 1244 pulmonologists. Respir Med. 2014;108(1):195-202. Epub 2013/12/24. doi: 10.1016/j.rmed.2013.11.017. PubMed PMID: 24361163.
20. Starfield B, Powe NR, Weiner JR, Stuart M, Steinwachs D, Scholle SH, et al. Costs vs quality in different types of primary care settings. JAMA. 1994;272(24):1903-8. Epub 1994/12/28. PubMed PMID: 7990241.
21. Schuster MA, McGlynn EA, Brook RH. How good is the quality of health care in the United States? Milbank Q. 1998;76(4):517-63, 09. Epub 1999/01/08. PubMed PMID: 9879302; PubMed Central PMCID: PMCPMC2751100.
22. Cabana MD, Rand CS, Powe NR, Wu AW, Wilson MH, Abboud PA, et al. Why don't physicians follow clinical practice guidelines? A framework for improvement. JAMA. 1999;282(15):1458-65. Epub 1999/10/27. doi: 10.1001/jama.282.15.1458. PubMed PMID: 10535437.
23. Cochrane LJ, Olson CA, Murray S, Dupuis M, Tooman T, Hayes S. Gaps between knowing and doing: understanding and assessing the barriers to optimal health care. J Contin Educ Health Prof. 2007;27(2):94-102. Epub 2007/06/20. doi: 10.1002/chp.106. PubMed PMID: 17576625.
24. Behr J. Guidelines or guidance for better idiopathic pulmonary fibrosis management? BMC Med. 2016;14:24. Epub 2016/02/11. doi: 10.1186/s12916-016-0567-9. PubMed PMID: 26860906; PubMed Central PMCID: PMCPMC4748484.
25. Garvey C, Novitch RS, Porte P, Casaburi R. Healing Pulmonary Rehabilitation in the United States. A Call to Action for ATS Members. Am J Respir Crit Care Med. 2019;199(8):944-6. Epub 2019/01/23. doi: 10.1164/rccm.201809-1711ED. PubMed PMID: 30667274; PubMed Central PMCID: PMCPMC6467299.
26. Johnston K, Grimmer-Somers K. Pulmonary rehabilitation: overwhelming evidence but lost in translation? Physiother Can. 2010;62(4):368-73. Epub 2011/09/03. doi: 10.3138/physio.62.4.368. PubMed PMID: 21886377; PubMed Central PMCID: PMCPMC2958065.
27. Olson AL, Swigris JJ, Lezotte DC, Norris JM, Wilson CG, Brown KK. Mortality from pulmonary fibrosis increased in the United States from 1992 to 2003. Am J Respir Crit Care Med. 2007;176(3):277-84. Epub 2007/05/05. doi: 10.1164/rccm.200701-044OC. PubMed PMID: 17478620.