Suspect pulmonary fibrosis
—a common threat across a wide range of interstitial lung diseases (ILDs), including connective tissue disease-associated interstitial lung diseases (CTD-ILDs). Pulmonary fibrosis can become a key driver of irreversible harm and early mortality, and calls for urgent identification and intervention.2–5
![Three patients holding lung scan results in their hands.](/sites/default/files/2021-08/P1%20.0-Home-Hero-max-width-1279px_0.png)
DISCOVER THE TRUE IMPACT OF FIBROTIC ILDs
![Scheme visualizing multidisciplinary team collaboration.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_1.png)
Diagnosis of ILD
![Spirometer and HRCT scan graphics.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9.png)
Early and regular monitoring
![Pie chart with random percentage values in the article teaser.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_3.png)
Critical threat of fibrotic ILDs
![Word cloud of terms associated with ILD identification.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_4.png)
Initial evaluation for ILDs
![HRCT image of Bronchiolectasis.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_5.png)
HRCT imaging of pulmonary fibrosis
![Blurred scheme of NON-IPF ILDs management algorithm.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_6.png)
Interventions for ILDs
![A female journalist interviewing Professor Walter Balle in his house.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_7.png)
SPIKES model: breaking bad news to your patients
![Arrow pointing down in the graph representing a poor survival following an acute exacerbation of IPF.](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_8.png)
Acute exacerbation of ILD
![Picture of three ILD patients (2 male and female).](/sites/default/files/2021-07/01-elements-placeholder-card-16-9_9.png)
ILD patient cases for evaluation and diagnosis
Footnotes
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CTD-ILD, connective tissue disease-associated interstitial lung disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis.
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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.
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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.
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Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013;10(4):362-370.
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Caban JJ, Yao J, Bagci U, Mollura DJ. Monitoring pulmonary fibrosis by fusing clinical, physiological, and computed tomography features. Conf Proc IEEE Eng Med Biol Soc. 2011;6216-6219.
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Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ, on behalf of the IPF Consensus Working Group. Eur Resp J. 2018;51:1800692.
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Theodore AC, Tseng C-H, Li N, Elashoff RM, Tashkin DP. Correlation of cough with disease activity and treatment with cyclophosphamide in scleroderma interstitial lung disease: findings from the Scleroderma Lung Study. Chest. 2012;142(3):614–621.
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Hoffmann-Vold AM, Fretheim H, Halse AK, et al. Tracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohort. Am J Respir Crit Care Med. 2019;200:1258–1266.
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Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. The Lancet Rheumatology. 2020b;2:e71–e83.
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Asano Y, Jinnin M, Kawaguchi Y, et al. Diagnostic criteria, severity classification and guidelines of systemic sclerosis: Guideline of SSc. J Dermatol. 2018;45, 633–691.
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Chaudhuri N, Spencer L, Greaves M, et al. A Review of the Multidisciplinary Diagnosis of Interstitial Lung Diseases: A Retrospective Analysis in a Single UK Specialist Centre. J Clin Med. 2016;5(66):1–9.
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Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.
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Wong AW, Ryerson C, Guler S. Progression of fibrosing interstitial lung disease. Respir Res. 2020:29;21(1):32.
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Brauner M, et al. Imagerie des pneumopathies infiltrantes diffuses. Press Med. 2010;39:73–84.
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Maher TM, Molina-Molina M, Russell AM, et al. Unmet needs in the treatment of idiopathic pulmonary fibrosis-insights from patient chart review in five European countries. BMC Pulm Med. 2017;17(1):124.
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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(7):537–539.
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Robalo-Cordeiro C, Campos P, Carvalho L, et al. Idiopathic pulmonary fibrosis in the era of antifibrotic therapy: Searching for new opportunities grounded in evidence. Rev Port Pneumol. 2017;23(5):287–293.
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Kolb M, Bondue B, Pesci A, et al. Acute exacerbations of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180071.
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Song JW, Hong S-B, Lim C-M, et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J. 2011;37(2):356–363.
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Song JW, Lee HK, Lee CK, et al. Clinical course and outcome of rheumatoid arthritis-related usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2013;30(2):103-112.
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Tomiyama F, Watanabe R, Ishii T, et al. High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis. Tohoku J. Exp. Med. 2016;239, 297–305.
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Okamoto M, Fujimoto K, Sadohara J, et al. A retrospective cohort study of outcome in systemic sclerosis-associated interstitial lung disease. Respiratory Investigation. 2016;54, 445–453.
Resources for patients
![Two seniors smiling together.](/sites/default/files/2021-07/image.png)
![Life with Pulmonary Fibrosis website logo.](/sites/default/files/2021-07/logo-primary_0.png)
![Scleroderma female patient looking into the window.](/sites/default/files/2021-07/image_0.png)
![More then scleroderma website logo.](/sites/default/files/2021-07/logo-primary_1.png)