What is pulmonary fibrosis?
Pulmonary fibrosis is a significant threat across a broad range of ILDs1–4
Pulmonary fibrosis occurs commonly in a wide range of interstitial lung diseases (ILDs), it can cause irreversible lung damage and is associated with increased mortality.1,3,5,6
WHAT HAPPENS IN PULMONARY FIBROSIS?2
In healthy lungs, oxygen is efficiently transferred from alveoli (air sacs) into the bloodstream.2
Pulmonary fibrosis is the build up of scar tissue in the lungs, leading to stiffness, as well as loss of function of alveolar (air sac) tissue, impairing gas exchange and making it difficult for your patients to breathe.2,7
Pulmonary fibrosis causes irreversible lung damage and sustained fibrosis leads to early mortality.3,5
ALTHOUGH INDIVIDUAL FIBROTIC ILDs ARE RARE, COLLECTIVELY, THEY AFFECT A CONSIDERABLE NUMBER OF PATIENTS6
Pulmonary fibrosis occurs in a wide range of ILDs8
Idiopathic pulmonary fibrosis (IPF)
Help your patients learn about their IPF with our IPF infographic
Connective tissue disease-associated interstitial lung diseases (CTD-ILDs)
Help your patients learn about their systemic sclerosis-associated ILD (SSc-ILD) and rheumatoid arthritis-associated ILD (RA-ILD) with our infographics
Hypersensitivity pneumonitis (HP)
Help your patients learn about their HP with our infographic below
Unclassifiable idiopathic interstitial pneumonias (IIP)
Idiopathic nonspecific interstitial pneumonia (iNSIP)
ILDs ARE CHARACTERIZED BY DAMAGE TO THE LUNG MEDIATED BY A COMBINATION OF LUNG INFLAMMATION AND FIBROSIS1,3,16–18
While each patient with ILD is unique, common pathogenic pathways to pulmonary fibrosis are shared.1,3,7,19
Patients with ILDs develop pulmonary fibrosis via common pathogenic pathways, irrespective of the underlying diagnosis or trigger.1,3,7,19,20
In the early stages of ILD pathogenesis, inflammation and fibrosis can coexist and lead to the development of pulmonary fibrosis.1,7,18,20–22
As ILD progresses, it may develop into progressive pulmonary fibrosis (PPF), leading to increased, self-sustaining fibrosis.7,18,22–24
Pulmonary fibrosis in ILD is driven by inflammation and fibrosis18,22,25,26
NURSES HAVE A KEY ROLE IN HELPING PATIENTS UNDERSTAND FIBROTIC ILDs AND THE PROGNOSIS27–32
Patients should be made aware that the disease course of ILD can be unpredictable, dependent on the ILD diagnosis and disease severity.1,7,33 ILD may become progressive despite standard of care, known as progressive pulmonary fibrosis (PPF) or progressive fibrosing ILD.7,8,19,33–35
Importantly, patients should understand that pulmonary fibrosis causes irreversible lung damage and sustained fibrosis may lead to early mortality.3,5
Nurses may also better educate caregivers as patients may struggle to explain their disease themselves.27
What other resources can help you support your patients in their journey with fibrotic ILD?
Progressive pulmonary fibrosis (PPF)
Impact of pulmonary fibrosis
- CO2, carbon dioxide; CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; HP, hypersensitivity pneumonitis; IIP, idiopathic interstitial pneumonia; ILD, interstitial lung disease; iNSIP, idiopathic non-specific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis; O2, oxygen; PPF, progressive pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease.
- 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;27:180076.
- Martinez FJ, Collard HR, Pardo A, et al. Idiopathic pulmonary fibrosis. Nat Rev Dis Primers. 2017;3:17074.
- 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. 2021;80:143–150.
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- Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692.
- Wijsenbeek M, Cottin V. Spectrum of fibrotic lung diseases. N Engl J Med. 2020;383:958–968.
- Kolb M, Vašáková M. The natural history of progressive fibrosing interstitial lung diseases. Respir Res. 2019;20:57.
- Raghu G, Remy-Jardin M, Richeldi L, 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:e18–e47.
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- Salisbury ML, Myers JE, Belloli EA, et al. 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.
- Duchemann B, Annesi-Maesano I, de Naurois CJ, et al. Prevalence and incidence of interstitial lung disease in a multi-ethnic country of Greater Paris. Eur Respir J. 2017;50:1602419.
- Nunes H, Bouvry D, Soler P, et al. Sarcoidosis. Orphanet J Rare Dis. 2007;2:46.
- Guler SA, Ryerson CJ. Unclassifiable interstitial lung disease: from phenotyping to possible treatments. Curr Opin Pulm Med. 2018;24(5):461–468
- Brown AW. Unclassifiable interstitial lung disease: time to shrink the black box. Ann Am Thorac Soc. 2018;15(7):806–807.
- Kinder BW, Collard HR, Koth L, et al. Idiopathic nonspecific interstitial pneumonia lung manifestation of undifferentiated connective tissue disease? Am J Respir Crit Care Med. 2007;176(7):691–697.
- Desai O, Winkler J, Minasyan M, et al. The role of immune and inflammatory cells in idiopathic pulmonary fibrosis. Front Med (Lausanne). 2018;5:43.
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- Wells AU, Denton CP. Interstitial lung disease in connective tissue disease – mechanisms and management. Nat Rev Rheumatol. 2014;10:728–39.
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- Duarte AC, Vinagre F, Soares J, et al. Antifibrotics in interstitial lung disease related to connective tissue diseases – a paradigm shift in treatment and outcome. Acta Rheumatol Port. 2019;44:161–162.
- Wollin L, Distler JHW, Redente EF, et al. Potential of nintedanib in treatment of progressive fibrosing interstitial lung diseases. Eur Respir J. 2019;54:1900161.
- Distler J, Györfi A-H, Ramanujam M, et al. Shared and distinct mechanisms of fibrosis. Nat Rev Rheumatol. 2019:15(12):705–730.
- Wongkarnjana A, Scallan C, Kolb MR. Progressive fibrosing interstitial lung disease: treatable traits and therapeutic strategies. Curr Opin Pulm Med. 2020;26:436–442.
- Saketkoo LA, Scholand MB, Lammi MR, Russell AM. Patient reported outcome measures in systemic sclerosis-related interstitial lung disease for clinical practice and clinical trials. J Scleroderma Relat Disord. 2020;5:48–60.
- Wollin L, Distler JHW, Denton CP, et al. Rationale for the evaluation of nintedanib as a treatment for systemic sclerosis–associated interstitial lung disease. J Scleroderma Relat Disord. 2019;4:212–218.
- Denton CP, Laird B, Moros L, Flores JLL. Things left unsaid: important topics that are not discussed between patients with systemic sclerosis, their carers and their healthcare professionals–a discourse analysis. Clin Rheumatol. 2021;40:1399–1407.
- Grewal JS, Ryerson CJ. A closer look at the multidisciplinary interstitial lung disease clinic: Who, what and how. Respirology. 2021;26:12–13.
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- Vega-Olivo M, Criner GJ. Idiopathic pulmonary fibrosis: A guide for nurse practitioners. Nurse Pract. 2018;43:48–54.
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28:180100.
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- Wijsenbeek M, Kreuter M, Olson A et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35:2015–2024.
- 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 Respir Res. 2017;4:e000212.
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