Prise en charge essentielle de la maladie pulmonaire interstitielle pour le prestataire de soins primaires
Résumé
Les maladies pulmonaires interstitielles (MPI) forment un groupe diversifié de troubles caractérisés par une inflammation et une fibrose du parenchyme pulmonaire. Bien qu’elles soient classées comme maladies rares, des données de plus en plus nombreuses suggèrent que les MPI sont plus répandues qu’on ne le pensait. Les patients présentent souvent des symptômes respiratoires tels qu’une dyspnée à l’effort, une toux persistante et de la fatigue. Toutefois, les patients asymptomatiques présentant des signes radiologiques fortuits (par exemple, des anomalies pulmonaires interstitielles) sont également
des cas fréquents. Le diagnostic repose sur la tomodensitométrie haute résolution (TDM-HR), les tests de la fonction pulmonaire et une évaluation clinique détaillée. Une consultation chez un pneumologue est importante pour assurer une prise en charge globale. L’évolution de la nomenclature des MPI, y compris la fibrose pulmonaire progressive, facilite la caractérisation de la maladie et la planification du traitement. Les stratégies de prise en charge ont recours aux corticostéroïdes et aux agents d’épargne des stéroïdes pour les sous-types inflammatoires, tandis que les traitements contre la fibrose (nintédanib, pirfénidone) sont utilisés pour les maladies fibrosantes et progressives. Les interventions non pharmacologiques, notamment la réadaptation pulmonaire, l’arrêt du tabac et la vaccination, sont essentielles pour améliorer les résultats des patients. Les fournisseurs de soins primaires jouent un rôle central dans le dépistage précoce de la maladie. Ils facilitent les tests de diagnostic, la prise en charge des affections concomitantes et la coordination des soins spécialisés. Cet article souligne l’importance d’un diagnostic rapide, du caractère évolutif des classifications et des nouveaux traitements, offrant ainsi un cadre de collaboration en vue d’optimiser les soins et les résultats dans le cadre des MPI.
Références
Cooley JC, Fernández Pérez ER. Are there over 200 distinct types of interstitial lung diseases? Respir Res. 2024;25(1):141. doi:10.1186/s12931-024-02734-0
Hopkins RB, Burke N, Fell C, Dion G, Kolb M. Epidemiology and survival of idiopathic pulmonary fibrosis from national data in Canada. Eur Respir J. 2016;48(1):187-195. doi:10.1183/13993003.01504-2015
Grant-Orser A, Liu Z, Fisher JH, Johannson KA. Epidemiology of interstitial lung disease: an administrative claims-based study in a universal health system. International Colloquium on Lung and Airway Fibrosis (ICLAF); Reykjavik, Iceland 2022.
Travis WD, Costabel U, Hansell DM, King TE, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733-748. doi:10.1164/rccm.201308-1483ST
Raghu G, Remy-Jardin M, Ryerson CJ, Myers JL, Kreuter M, Vasakova M, et al. Diagnosis of hypersensitivity pneumonitis in adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69. doi:10.1164/rccm.202005-2032ST
Cottin V, Hirani NA, Hotchkin DL, Nambiar AM, Ogura T, Otaola M, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150). doi:10.1183/16000617.0076-2018
Grant-Orser A, Pooler C, Archibald N, Fell C, Ferrara G, Johannson KA, et al. The diagnostic pathway for patients with interstitial lung disease: a mixed-methods study of patients and physicians. BMJ Open Respir Res. 2024;11(1). doi:10.1136/bmjresp-2024-002333
Hoyer N, Prior TS, Bendstrup E, Wilcke T, Shaker SB. Risk factors for diagnostic delay in idiopathic pulmonary fibrosis. Respir Res. 2019;20(1):103. doi:10.1186/s12931-019-1076-0
Rahman KM, Samaria J. Diagnostic delay and misdiagnosis in interstitial lung disease (ILD) at primary health care level. European Respiratory Journal. 2016;48. Doi:10.1183/13993003.CONGRESS-2016.PA861
Auld SC, Sheshadri A, Alexander-Brett J, Aschner Y, Barczak AK, Basil MC, et al. Postinfectious pulmonary complications: establishing research priorities to advance the field: an Official American Thoracic Society Workshop Report. Ann Am Thorac Soc. 2024;21(9):1219-1237. doi:10.1513/AnnalsATS.202406-651ST
Kaul B, Cottin V, Collard HR, Valenzuela C. Variability in global prevalence of interstitial lung disease. Front Med (Lausanne). 2021;8:751181. doi:10.3389/fmed.2021.751181
Mayes MD, Lacey JV, Beebe-Dimmer J, Gillespie BW, Cooper B, Laing TJ, et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum. 2003;48(8):2246-2255. doi:10.1002/art.11073
Hunninghake GM, Quesada-Arias LD, Carmichael NE, Martinez Manzano JM, Poli De Frías S, Baumgartner MA, et al. Interstitial lung disease in relatives of patients with pulmonary fibrosis. Am J Respir Crit Care Med. 2020;201(10):1240-1248. doi:10.1164/rccm.201908-1571OC
Grant-Orser A, Min B, Elmrayed S, Podolanczuk AJ, Johannson KA. Prevalence, risk factors, and outcomes of adult interstitial lung abnormalities: a systematic review and meta-analysis. Am J Respir Crit Care Med. 2023;208(6):695-708. doi:10.1164/rccm.202302-0271OC
Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Screening and Monitoring of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis Rheumatol. 2024;76(8):1201-1213. doi:10.1002/art.42860
Jee AS, Sheehy R, Hopkins P, Corte TJ, Grainge C, Troy LK, et al. Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: a position statement from the Thoracic Society of Australia and New Zealand. Respirology. 2021;26(1):23-51. doi:10.1111/resp.13977
Dyer DS, White C, Conley Thomson C, Gieske MR, Kanne JP, Chiles C, et al. A quick reference guide for incidental findings on lung cancer screening CT examinationsJ AM Coll Radiol. 2023;20(2):162-172. doi:10.1016/j.jacr.2022.08.009
Oldham JM, Adegunsoye A, Khera S, Lafond E, Noth I, Strek ME, et al. Underreporting of interstitial lung abnormalities on lung cancer screening computed tomography. Ann Am Thorac Soc. 2018;15(6):764-766. doi:10.1513/AnnalsATS.201801-053RL
Balata H, Punjabi A, Chaudhuri N, Greaves M, Yorke J, Booton R, et al. The detection, assessment and clinical evolution of interstitial lung abnormalities identified through lung cancer screening. ERJ Open Res. 2023;9(3). doi:10.1183/23120541.00632-2022
Hatabu H, Hunninghake GM, Richeldi L, Brown KK, Wells AU, Remy-Jardin M, et al. Interstitial lung abnormalities detected incidentally on CT: a Position Paper from the Fleischner Society. Lancet Respir Med. 2020;8(7):726-737. doi:10.1016/S2213-2600(20)30168-5
Moran-Mendoza O, Ritchie T, Aldhaheri S. Fine crackles on chest auscultation in the early diagnosis of idiopathic pulmonary fibrosis: a prospective cohort study. BMJ Open Respir Res. 2021;8(1). doi:10.1136/bmjresp-2020-000815
Sgalla G, Simonetti J, Di Bartolomeo A, Magrì T, Iovene B, Pasciuto G, et al. Reliability of crackles in fibrotic interstitial lung disease: a prospective, longitudinal study. Respir Res. 2024;25(1):352. doi:10.1186/s12931-024-02979-9
King TE, Tooze JA, Schwarz MI, Brown KR, Cherniack RM. Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med. 2001;164(7):1171-1181. doi:10.1164/ajrccm.164.7.2003140
Pereira CAC, Soares MR, Boaventura R, Castro MDC, Gomes PS, Gimenez A, et al. Squawks in interstitial lung disease prevalence and causes in a cohort of one thousand patients. Medicine (Baltimore). 2019;98(29):e16419. doi:10.1097/MD.0000000000016419
van Manen MJG, Vermeer LC, Moor CC, Vrijenhoeff R, Grutters JC, Veltkamp M, et al. Clubbing in patients with fibrotic interstitial lung diseases. Respir Med. 2017;132:226-231. doi:10.1016/j.rmed.2017.10.021
Suliman YA, Dobrota R, Huscher D, Nguyen-Kim TD, Maurer B, Jordan S, et al. Brief report: pulmonary function tests: high rate of false-negative results in the early detection and screening of scleroderma-related interstitial lung disease. Arthritis Rheumatol. 2015;67(12):3256-3261. doi:10.1002/art.39405
Kolb M, Richeldi L, Behr J, Maher TM, Tang W, Stowasser S, et al. Nintedanib in patients with idiopathic pulmonary fibrosis and preserved lung volume. Thorax. 2017;72(4):340-346. doi:10.1136/thoraxjnl-2016-208710
Johannson KA, Kolb M, Fell CD, Assayag D, Fisher J, Churg A, et al. Evaluation of patients with fibrotic interstitial lung disease: A Canadian Thoracic Society position statement. Canadian Journal of Respiratory, Critical Care, and Sleep Medicine. 2017;1(3):133-141. doi: 10.1080/24745332.2017.1359056
Fidler L, Doubelt I, Kandel S, Fisher JH, Mittoo S, Shapera S. Screening for myositis antibodies in idiopathic interstitial lung disease. Lung. 2019;197(3):277-284. doi:10.1007/s00408-019-00212-9
Barnes H, Elmrayed S, Barber CM, Feary J, Lee CT, Gandhi S, et al. Scoping review of exposure questionnaires and surveys in interstitial lung disease. BMJ Open Respir Res. 2024;11(1). doi:10.1136/bmjresp-2023-002155
Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):e18-e47. doi:10.1164/rccm.202202-0399ST
du Bois RM, Weycker D, Albera C, Bradford WZ, Costabel U, Kartashov A, et al. Forced vital capacity in patients with idiopathic pulmonary fibrosis: test properties and minimal clinically important difference. Am J Respir Crit Care Med. 2011;184(12):1382-1389. doi:10.1164/rccm.201105-0840OC
Alfieri V, Crisafulli E, Visca D, Chong WH, Stock C, Mori L, et al. Physiological predictors of exertional oxygen desaturation in patients with fibrotic interstitial lung disease. Eur Respir J. 2020;55(2). doi:10.1183/13993003.01681-2019
Travis WD, Hunninghake G, King TE, Lynch DA, Colby TV, Galvin JR, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008;177(12):1338-1347. doi:10.1164/rccm.200611-1685OC
Walsh SLF, Maher TM, Kolb M, Poletti V, Nusser R, Richeldi L, et al. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study. Eur Respir J. 2017;50(2). doi:10.1183/13993003.00936-2017
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):557-565. doi:10.1016/S2213-2600(16)30033-9
Foundation CPF. Find an ILD Respirologist in Canada 2024. [cited 27 February 2025] Available from: https://cpff.ca/read-next/2-2-read-next-diagnosis/ild-respirologists-across-canada/.
Cutting CC, Bowman WS, Dao N, Pugashetti JV, Garcia CK, Oldham JM, et al. Family history of pulmonary fibrosis predicts worse survival in patients with interstitial lung disease. Chest. 2021;159(5):1913-1921. doi:10.1016/j.chest.2021.01.026
Cottin V, Selman M, Inoue Y, Wong AW, Corte TJ, Flaherty KR, et al. Syndrome of combined pulmonary fibrosis and emphysema: An Official ATS/ERS/JRS/ALAT Research Statement. Am J Respir Crit Care Med. 2022;206(4):e7-e41. doi:10.1164/rccm.202206-1041ST
Zhang D, Adegunsoye A, Oldham JM, Kozlitina J, Garcia N, Poonawalla M, et al. Telomere length and immunosuppression in non-idiopathic pulmonary fibrosis interstitial lung disease. Eur Respir J. 2023. doi:10.1183/13993003.00441-2023
Peljto AL, Zhang Y, Fingerlin TE, Ma SF, Garcia JG, Richards TJ, et al. Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA. 2013;309(21):2232-2239. doi:10.1001/jama.2013.5827
Fainberg HP, Moodley Y, Triguero I, Corte TJ, Sand JMB, Leeming DJ, et al. Cluster analysis of blood biomarkers to identify molecular patterns in pulmonary fibrosis: assessment of a multicentre, prospective, observational cohort with independent validation. Lancet Respir Med. 2024;12(9):681-692. doi:10.1016/S2213-2600(24)00147-4
Johnson SR, Bernstein EJ, Bolster MB, Chung JH, Danoff SK, George MD, et al. 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases. Arthritis Rheumatol. 2024;76(8):1182-1200. doi:10.1002/art.42861
Morisset J, Johannson KA, Vittinghoff E, Aravena C, Elicker BM, Jones KD, et al. Use of mycophenolate mofetil or azathioprine for the management of chronic hypersensitivity pneumonitis. Chest. 2017;151(3):619-625. doi:10.1016/j.chest.2016.10.029
Richeldi L, Kolb M, Jouneau S, Wuyts WA, Schinzel B, Stowasser S, et al. Efficacy and safety of nintedanib in patients with advanced idiopathic pulmonary fibrosis. BMC Pulm Med. 2020;20(1):3. doi:10.1186/s12890-019-1030-4
King TE, 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):2083-2092. doi:10.1056/NEJMoa1402582
Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718-1727. doi:10.1056/NEJMoa1908681
Wu Z, Banya W, Chaudhuri N, Jakupovic I, Maher TM, Patel B, et al. PAciFy Cough-a multicentre, double-blind, placebo-controlled, crossover trial of morphine sulphate for the treatment of pulmonary fibrosis cough. Trials. 2022;23(1):184. doi:10.1186/s13063-022-06068-4
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. doi:10.1164/rccm.201807-1255ST
Salisbury ML, Myers JL, Belloli EA, Kazerooni EA, Martinez FJ, Flaherty KR. Diagnosis and treatment of fibrotic hypersensitivity pneumonia. Where we stand and where we need to go. Am J Respir Crit Care Med. 2017;196(6):690-699. doi:10.1164/rccm.201608-1675PP
JafariNezhad A, YektaKooshali MH. Lung cancer in idiopathic pulmonary fibrosis: a systematic review and meta-analysis. PLoS One. 2018;13(8):e0202360. doi:10.1371/journal.pone.0202360
Visca D, Mori L, Tsipouri V, Fleming S, Firouzi A, Bonini M, et al. Effect of ambulatory oxygen on quality of life for patients with fibrotic lung disease (AmbOx): a prospective, open-label, mixed-method, crossover randomised controlled trial. Lancet Respir Med. 2018;6(10):759-770. doi:10.1016/S2213-2600(18)30289-3
Dowman L, Hill CJ, May A, Holland AE. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev. 2021;2(2):CD006322. doi:10.1002/14651858.CD006322.pub4
Bischoff KE, Choi S, Su A, Cohen E, O'Riordan DL, Oettel E, et al. Better together: a mixed-methods study of palliative care co-management for patients with interstitial lung disease. J Palliat Med. 2021;24(12):1823-1832. doi:10.1089/jpm.2020.0787
Bekelman DB, Feser W, Morgan B, Welsh CH, Parsons EC, Paden G, et al. Nurse and social worker palliative telecare team and quality of life in patients with COPD, heart failure, or interstitial lung disease: The ADAPT Randomized Clinical Trial. JAMA. 2024;331(3):212-223. doi:10.1001/jama.2023.24035
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