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It's Pulmonary Fibrosis Awareness Month – September 2023

Progressive fibrosis is associated with worsened respiratory symptoms, irreversible lung function decline, reduced health-related quality of life, and early mortality.1

Discover more about signs and symptoms of ILD

SUSPECT PULMONARY FIBROSIS

—a common threat for many patients with interstitial lung disease (ILD),2–8 associated with irreversible lung function decline and early mortality, and calls for early recognition and timely referral to a lung specialist1,4,5,9

suspect pulmonary fibrosis banner

DISCOVER WHY RECOGNITION AND REFERRAL OF FIBROTIC ILD IS IMPORTANT

LUNG AUSCULTATION SOUNDS AND FLOW-VOLUME LOOPS

Improve your ability to detect fibrotic ILDs using the sounds and flow-volume loops of lung auscultation.

‘Velcro-like’ crackles are valuable in identifying fibrotic ILD early.13,14

Download interactive slideshow
LUNG AUSCULTATION SOUNDS AND FLOW-VOLUME LOOPS
Footnotes

ILD, interstitial lung disease.

  1. Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: A double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
  2. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019;20(1):13.
  3. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718-1727.
  4. Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20(1):57.
  5. Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36(7):1518–1531.
  6. Spagnolo P, Distler O, Ryerson CJ, et al. Mechanisms of progressive fibrosis in connective tissue disease (CTD)-associated interstitial lung diseases (ILDs). 
    Ann Rheum Dis. 2020;80:143–150.
  7. Volkmann E, Varga J. Emerging targets of disease-modifying therapy for systemic sclerosis. Nat Rev Rheumatol. 2019;15:208–224.
  8. Wijsenbeek MS, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 
    2019:1–10.
  9. Zibrak JD, Price D. Interstitial lung disease: raising the index of suspicion in primary care. NPJ Prim Care Respir Med. 2014;24:14054.
  10. Swigris JJ, Brown KK, Abdulqawi R, et al. Patients’ perceptions and patient reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180075.
  11. Wuyts WA, Papiris S, Manali E, et al. The burden of progressive fibrosing interstitial lung disease: a DELPHI approach. Adv Ther. 2020;37(7):3246–3264.
  12. Mittoo S, Frankel S, LeSage D, et al. Patient perspectives in OMERACT provide an anchor for future metric development and improved approaches to healthcare delivery in connective tissue disease related interstitial lung disease (CTD-ILD). Curr Respir Med Rev. 2015;11(2):175–183.
  13. Sgalla G, Walsh SLF, Sverzellati N, et al. “Velcro-type” crackles predict specific radiologic features of fibrotic interstitial lung disease. BMC Pulm Med. 2018;18(1):103.
  14. Cottin V, Cordier J. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J. 2012;40(3):519–521.
  15. Molina-Molina M, Aburto M, Acosta O, et al. Importance of early diagnosis and treatment in idiopathic pulmonary fibrosis. Exp Rev Resp Med. 2018;12:537–539.
  16. Manfredi A, Cassone G, Cerri S, et al. Diagnostic accuracy of a velcro sound detector (VECTOR) for interstitial lung disease in rheumatoid arthritis patients: the InSPIRAtE validation study (INterStitial pneumonia in rheumatoid ArThritis with an electronic device). BMC Pulm Med. 2019;19(1):111.
  17. Ryerson CJ, Urbania TH, Richeldi L, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42:750–757.
  18. Hyldgaard C, Hilberg O, Pedersen AB, et al. A population-based cohort study of rheumatoid arthritis-associated interstitial lung disease: comorbidity and mortality. Ann Rheum Dis. 2017;76(10):1700–1706.
  19. Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis. Am J Respir Crit Med.1994;149(6):1583–1590.
  20. Mapel DW, Hunt WC, Utton R, et al. Coultas DB. Idiopathic pulmonary fibrosis: survival in population based and hospital based cohorts. Thorax. 1998;53(6):469–476.
  21. Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a population-based cohort study. Chest. 1998;113(2):396–400.
  22. Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 
    2008;177(11):1248–1254.
  23. Moore OA, Goh N, Corte T, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52(1):155–160.
  24. Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–1328.
  25. Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: a population-based study. Arthritis Rheum. 2010;62(6):1583–1591.
  26. Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax. 2018;73(4):391–392.
  27. Vourlekis JS, Schwarz MI, Cherniack RM, et al. The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis. Am J Med. 
    2004;116(10):662–668.
  28. Cottin V, Hirani N, Hotchkin D, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.
  29. Demedts M, Wells AU, Antó JM, et al. Interstitial lung diseases: an epidemiological overview. Eur Respir J. 2001;18(suppl 32):2s–16s.
  30. Ryu JH, Daniels CE, Hartman TE, Yi ES. Diagnosis of interstitial lung diseases. Mayo Clin Proc. 2007;82(8):976–986.

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