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INTRODUCTION

Respiratory Disease

The term interstitial lung disease (ILD) encompasses a large group of more than 200 pulmonary disorders, with the most being classified as rare. Early and accurate diagnosis can be challenging, and it is difficult to predict disease progression. IPF is the most common ILD.16

Idiopathic Pulmonary Fibrosis (IPF)

  • IPF is relentless, progressive, and deadly.1

  • IPF is progressive and debilitating with a median survival of 2 to 5 years after diagnosis.1-3

  • It is therefore extremely important that IPF is diagnosed early and accurately, as this would be the first step in helping patients manage this debilitating disease.4

Systemic Sclerosis associated Interstitial lung disease (SSc-ILD)

  • SSc is a rare and heterogeneous connective tissue disease characterised by fibrosis affecting the skin and multiple organs, including the lungs, and Interstitial lung disease (ILD) is a common manifestation of SSc.5-8

  • Furthermore, ILD is the leading cause of mortality in SSc, responsible for up to 35% of SSc-related deaths.9,10

Other chronic fibrosing ILDs

  • As many as 18% to 32% of patients with non-IPF ILDs are estimated to be at risk for developing a progressive fibrosing phenotype, and these patients follow a similar disease course and prognosis as that observed with IPF.11-14

At Boehringer Ingelheim Specialty Care, we offer a new hope for patients diagnosed with IPF, SSc-ILD or other ILDs with a progressive phenotype 15. Take courage and face Pulmonary Fibrosis Head On!

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Reference:

  1. Ley B, Collard HR, King TE Jr. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431-440. doi:10.1164/rccm.201006-0894CI.

  2. Adamali HI, Anwar MS, Russell A-M, Egan JJ. Non-pharmacological treatment of idiopathic pulmonary fibrosis. Curr Respir Care Rep. 2012;1(4):208-215. doi:10.1007/s13665-012-0031-z. 

  3. US Food and Drug Administration. The Voice of the Patient: Idiopathic Pulmonary Fibrosis. Washington, DC: US Food and Drug Administration; March 2015.

  4. Oldham JM, Noth I. Idiopathic pulmonary fibrosis: early detection and referral. Respir Med. 2014;108(6):819-829. doi:10.1016/j.rmed.2014.03.008.

  5. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir Res. 2019;20(13):1-10. doi:10.1186/s12931-019-0980-7. 

  6. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685-1699. doi:10.1016/S0140-6736(17)30933-9.

  7. Walker UA, Tyndall A, Czirjak L, et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2007;66(6):754-763. doi:10.1136/ard.2006.062901. 

  8. Jaeger VK, Wirz EG, Allanore Y, et al. Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study. PLoS One. 2016;11(10):e0163894. doi:10.1371/journal.pone.0163894.

  9. Tyndall AJ, Bannert B, Vonk M, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69(10):1809-1815. doi:10.1136/ard.2009.114264. 

  10. Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940-944. doi:10.1136/ard.2006.066068.

  11. Wijsenbeek M, Kreuter M, Fischer A, et al. Non-IPF progressive fibrosing interstitial lung disease (PF-ILD): the patient journey. Am J Respir Crit Care Med. 2018;197:1-2.

  12. Flaherty KR, Wells AU, Cottin V, et al. Nintedanib in progressive fibrosing interstitial lung diseases. N Engl J Med. 2019;381(18):1718-1727. doi:10.1056/NEJMoa1908681.

  13. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180076.

  14. Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27:180071 doi:/10.1183/16000617.0071-2018.

  15. OFEV® Professional information leaflet; Ingelheim Pharmaceuticals (Pty) Ltd 31 July 2021.

  16. Vincent Cottin Nikhil A. Hirani, David L. Hotchkin, Anoop M. Nambiar, Takashi Ogura, María Otaola, Dirk Skowasch, Jong Sun Park, Hataya K. Poonyagariyagorn, Wim Wuyts, Athol U. Wells European Respiratory Review. 2018;27:180076. DOI: 10.1183/16000617.0076-2018

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