This website is owned and developed by Boehringer Ingelheim and contains promotional information.
This website is intended for UK and Ireland Healthcare Professionals only. If you are not a Healthcare Professional in the UK or Ireland please click here.
Click here for OFEV® (nintedanib) UK prescribing information. Find adverse event reporting information at the bottom of this page.
Click here for OFEV® (nintedanib) Ireland prescribing information. Find adverse event reporting information at the bottom of this page.

ILDs are a diverse group of rare diseases

Interstitial lung diseases (ILDs) are a diverse and complex group of rare diseases. Each ILD has its own characteristic aetiology (where known) and prognosis.1 Classifications are usually based on available knowledge of the disease mechanisms and the clinical, radiological and histological presentations.1-5

Significant overlap can exist between ILDs and the ability to discern between them using current investigatory techniques, such as high resolution computed tomography (HRCT), can make diagnosis complex.2,4,5 Given the fibrotic potential of many of these ILDs,7 significant vigilance and intuition is needed on the part of treating and referring physicians to limit loss of function.

There are five main classifications of ILD, each grouped according to their shared characteristics. Download an overview of the classifications here.

Significant vigilance and intuition is needed on the part of treating and referring physicians to limit loss of function and early mortality.5

Explore ILDs

Idiopathic interstitial pneumonias (IIPs)

Cause: unknown aetiology8

Characterised by: diagnosis of exclusion that results in a highly heterogenous group of ILDs with varying degrees of inflammation and fibrosis8 – idiopathic pulmonary fibrosis (IPF) being the most prevalent form7

There are nine subtypes of IIP, each with their own specific risk factors1,9,10

Percentage graph shows subtypes of Idiopathic Interstitial Pneumonias each with their own specific risk factors - 55% Idiopathic Pulmonary Fibrosis/ 25% Idiopathic non-specific interstitial pneumonia/ 10% respiratory bronchiolitis associated interstitial lung disease/ 5% Desquamative interstitial pneumonia/ 3% Cryptogenic Organising Pneumonia/ 1% Acute interstitial Pneumonia/ 1% Idiopathic lymphoid interstitial pneumonia/ <1% Idiopathic pleuroparenchymal fibroelastosis

10% of IIPs are unclassifiable, likely due to surgical risk preventing biopsy or conflicting clinical, radiological or histopathological data1,9,11-13

Pulmonary fibrosis: IPF is the most common* and fibrotic form of IIP with 100% of patients developing progressive
fibrotic ILD7,14

Clinical course: highly variable between IIPs with IPF having the worst prognosis of 2-5 years, driven by fibrotic progression8,15

*Based on a review of 848 cases from physicians and a social security database in a county in Paris

The fibrotic threat to lung function42-46

Fibrosis is a complex response to injury involving many interlinked signalling pathways and mediators, such as chemokines, thrombin, endothelin-1 and many growth factors.42-46 Imbalances in these signalling pathways propagate a self-sustaining mechanism of fibrosis with potential to cause irreversible scarring to lung parenchyma.42-46 Uncontrolled, this leads to progressive functional decline and increased morbidity and mortality.6

Although it is still unclear why some patients develop a chronic self-sustaining fibrotic form while others do not, it is known that the threat is associated with repeated or sustained tissue injury and is common to patients across different ILDs.42-46 This phenotypic expression is termed chronic progressive fibrotic ILD.47

Imbalances in these signalling pathways propagate a self-sustaining mechanism of fibrosis.42-46

Pathophysiology of pulmonary fibrosis

Watch to see the destructive process of pulmonary fibrosis

Video still: Interstitial lung diseases (ILDs) and progressive fibrotic phenotype video / A row of transparent people with their lungs visible

WATCH: Interstitial lung diseases (ILDs) and progressive fibrotic phenotype

Video still: Pulmonary fibrosis and abnormal wound healing video

WATCH: Pulmonary fibrosis and abnormal wound healing

Video still: Profibrotic environments leading to tissue injury video

WATCH: Profibrotic environments leading to tissue injury

Video still: Pulmonary fibrosis has an independent, self-sustaining mechanism video shows circular flow graph self-sustaining fibrotic cycle/ matrix stiffness/ tissue pressure/ hypoxia

WATCH: Pulmonary fibrosis has an independent, self-sustaining mechanism

What happens when progressive pulmonary fibrosis starts
to drive patient decline?

Find out more

  1. ATS/ERS. Am J Respir Crit Care Med 2002;165:277-304.
  2. Raghu G et al. Am J Respir Crit Care Med 2011;183:788-824.
  3. Walsh SLF. Eur Respir Rev 2017;26:170002.
  4. Lynch DA et al. Lancet Respir Med 2018;6:138-53.
  5. Maher TM. Immunol Allergy Clin North Am 2012;32:453-72.
  6. Ley B et al. Am J Respir Crit Care Med 2011;183(4):431-440.
  7. Olson A et al. European Respiratory Journal 2018;52:PA3030.
  8. Lee J. Available at: https://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/overview-of-idiopathic-interstitial-pneumonias (Accessed July 2023).
  9. Travis WD et al. Am J Respir Crit Care Med 2013;188:733-48.
  10. European Respiratory Society. Interstitial Lung Diseases. In: The European Lung White Book: Respiratory Health and Disease in Europe. 2nd Ed. Sheffield: Eur Respir Soc; 2013.
  11. Ryerson CJ et al. Eur Respir J 2013;42:750-757.
  12. Hyldgaard C et al. Respirology 2017;22:494–500.
  13. De Sadeleer LJ et al. Chest 2018;153:1416-1423.
  14. Duchemann B et al. Eur Respir J 2017;50;pii:1602419.
  15. Fujimoto H et al. Clin Med Insights Circ Respir Pulm Med 2016;8(9):179-185.
  16. Koo S-M et al. Kor J Intern Med. 2017;32(4):600-610.
  17. Fischer A and Richeldi L. Semin Respir Crit Care Med 2014;35:159-165.
  18. Wallace B et al. Curr Opin Rheumatol 2016;28(3):236-245.
  19. Witt LJ et al. Clin Pulm Med. 2016;23(5):218–226.
  20. Flaherty KR et al. N Engl J Med 2019;381(18):1718-1727.
  21. Cottin V et al. Eur Respir Rev 2018;27(150):180076.
  22. Strange C et al. Clin Chest Med 2004;25(3):549-559.
  23. Selman M et al. Am J Respir Crit Care Med 2012;186:314-324.
  24. Vasakova M et al. Am J Respir Crit Care Med 2017;196:680-689.
  25. Salisbury ML et al. Am J Respir Crit Care Med 2017;196:690-699.
  26. Fernández Pérez ER et al. Ann Am Thorac Soc 2018;15:460-469.
  27. American Thoracic Society. Am J Respir Crit Care Med 1999;160(2):736-755.
  28. Schupp JC et al. Eur Respir J 2018;51(1):1700991.
  29. Baughman RP et al. Ann Am Thorac Soc 2016;13:1244-1252.
  30. Kearney GD et al. Respir Med 2019;149:30-35.
  31. Ianuzzi MC et al. Available at: https://www.merckmanuals.com/professional/pulmonary-disorders/sarcoidosis/sarcoidosis (Accessed July 2023).
  32. Coker RK. Ther Clin Risk Manag 2009;5(3):575-584.
  33. Beegle SH et al. Drug Des Devel Ther 2013;7:325-338.
  34. Patterson KC et al. Ann Am Thorac Soc 2013;10(4):362-370.
  35. Leung CC et al. Lancet 2012;379:2008-2018.
  36. Mayer A and Hamzeh N. Curr Opin Pulm Med 2015;21:178-84.
  37. Glazer CS. Clin Pulm Med 2011;18:20-28.
  38. Schwaiblmair M et al. Open Respir Med J. 2012;6:63-74.
  39. Lee J. Lymphangioleiomyomatosis. The Merck Manual Online. 2018. Available at: https://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/lymphangioleiomyomatosis (Accessed July 2023).
  40. Hancock E et al. Respir Med. 2002;96(1):1-6.
  41. Tazi A. Eur Respir J. 2006;27:1272-1285.
  42. Bagnato G and Harrari S. Eur Respir Rev 2015;25:102-114.
  43. Liu F et al. J Cell Biol 2010;190:693-706.
  44. Barkauskas CE and Noble PW. Am J Physiol Cell Physiol 2014;306:C987-C996.
  45. Parker MW et al. J Clin Invest 2014;124:1622-1635.
  46. Pechkovsky DV et al. Clin Immunol 2010;137:89-101.
  47. OFEV® 100 mg and 150 mg soft capsules Summary of Product Characteristics. Boehringer Ingelheim.

Adverse events should be reported.

UK: Reporting forms and information can be found at www.mhra.gov.uk/yellowcard. Adverse events should also be reported to Boehringer Ingelheim Drug Safety on 0800 328 1627 (freephone) or by email to PV_local_uk_ireland@boehringer-ingelheim.com.

IE: Reporting forms and information can be found at www.hpra.ie/homepage/about-us/report-an-issue. Adverse events should also be reported to Boehringer Ingelheim Drug Safety on 01 291 3960 or by email to PV_local_uk_ireland@boehringer-ingelheim.com.

OFEV® is indicated in adults for the treatment of idiopathic pulmonary fibrosis (IPF) and for the treatment of other chronic fibrosing interstitial lung diseases (ILDs) with a progressive phenotype. The recommended dose is one 150 mg capsule taken twice daily.47

PC-GB-104224 | October 2021