Common to many ILDs, pulmonary fibrosis is characterised by the often chronic and irreversible scarring of lung tissue.2,3
ILDs are a diverse group of more than 200 heterogeneous lung disorders, mostly classified as rare or only infrequently seen in clinical practice.1,5,6
Patients with CTDs are at risk of developing an ILD.1,5
Crosses diverse ILDs:
Including many CTD-ILDs:
See what Vincent Cottin, Professor of Respiratory Medicine, has to say about the
threat of pulmonary fibrosis across a range of ILDs
Development of ILD-associated pulmonary fibrosis is thought to be mediated through the migration and proliferation of fibroblasts and their differentiation into myofibroblasts9,11
As fibroblasts and myofibroblasts invade the pulmonary tissue, they continually synthesise and release collagen and extracellular matrix protein9,11
Excessive extracellular matrix protein remodelling leads to irreversible distortion of lung architecture, compromising pulmonary capacity, function, and gas exchange9,11
Watch to see the destructive process of pulmonary fibrosis
Pulmonary fibrosis has a devastating impact across CTDs, including SSc-ILD.
See what you can do to make a difference.
Flaherty KR, Brown KK, Wells AU, et al. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of ... in patients with progressive fibrosing interstitial lung disease. BMJ Open Resp Res. 2017;4(1):e000212.
Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013;10(4):362-370.
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.
Wells AU, Brown KK, Flaherty KR, Kolb M, Thannickal VJ, on behalf of the IPF Consensus Working Group. Eur Resp J. 2018;51:1800692.
Demedts M, Wells AU, AntÓ JM, et al. Interstitial lung diseases: an epidemiological overview. Eur Respir J Suppl. 2001;18(suppl32):2s-16s.
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.
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.
Koo S-M, Uh S-T. Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist's point of view. Korean J Intern Med. 2017;32(4):600-610.
Selman M, King TE Jr, Pardo A. Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy. Ann Intern Med. 2001;134(2):136-151.
Bagnato G, Harari S. Cellular interactions in the pathogenesis of interstitial lung diseases. Eur Respir Rev. 2015;24(135):102-114.
Raghu G, Collard HR, Egan JJ, et al; on behalf of the ATS/ERS/JRS/ALAT Committee on Idiopathic Pulmonary Fibrosis. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.
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