Chronic progressive fibrotic ILD
What is chronic progressive fibrotic ILD?
Chronic progressive fibrotic interstitial lung disease (ILD) is a clinically challenging development in the course of many ILDs.1 Although classed as rare diseases, ILDs impact a wide range of patients, including both men and women.2 Damage caused by fibrosis leads to irreversible scarring of the underlying lung tissue, reducing lung function, worsening breathlessness, affecting patients' livelihoods and resulting in death.2
Despite the individual pathologies and clinical pathways that characterise different ILDs, many patients converge on a common phenotype, described as chronic progressive fibrotic ILD.3–5 For these patients, chronic progressive fibrotic ILD has become the primary driver of disease progression and clinical decline, irrespective of the originating pathology or stimulus.
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Which ILDs can become progressive?
An estimated 13–40% of patients with non-idiopathic pulmonary fibrosis (IPF) ILDs are at risk of developing a chronic progressive fibrotic phenotype.1,6
Non-IPF ILDs that may become progressive include:1
- Rheumatoid arthritis-ILD (RA-ILD)
- Systemic sclerosis-ILD (SSc-ILD)
- Mixed connective tissues disease-ILD (mCTD-ILD)
- Hypersensitivity pneumonitis (HP)
- Idiopathic non-specific interstitial pneumonia (iNSIP)
- Unclassifiable idiopathic interstitial pneumonia (nIIP)
- Sarcoidosis ILD
Identifying chronic progressive fibrotic ILD
The clinical characteristics of the chronic progressive fibrotic phenotype were documented in the landmark INBUILD® study, which looked into the treatment of chronic progressive fibrotic ILD patients with OFEV® (nintedanib). In the study, patients were screened for the extent of their fibrosis and markers of progression. The INBUILD® study offers insight into the identification, management and treatment needs of this vulnerable patient population.3
Commitment to progress
Thanks to the commitment of the ILD community and Boehringer Ingelheim, significant progress has been made for these rare diseases over the past decade. Awareness, knowledge and understanding of the role fibrosis plays in disease progression has grown. Today, the effect of OFEV® on fibrosis in diseases other than IPF has introduced opportunities to slow the rate of disease progression in more patients with chronic progressive fibrotic ILDs.3,4,7 By raising awareness of the chronic progressive fibrotic phenotype and the impact this type of progressive pulmonary fibrosis has on ILD patients, we hope to help improve the diagnosis, care and outcomes for patients living with these diseases.
References
1.
Olson A et al. European Respiratory Society International Congress, Paris, France, 15−19 September 2018; PA3030.
2.
Ley B et al. Am J Respir Crit Care Med 2011;183(4):431–440.
3.
Flaherty KR et al. N Engl J Med 2019;381(18):1718–1727.
4.
Richeldi L et al. N Engl J Med 2014;370(22):2071–2082.
5.
Cottin V et al. Eur Respir J 2012;40(3):519–521.
6.
Wijsenbeek M et al. Am J Respir Crit Care Med 2018;197:1–2.
7.
OFEV® 100 mg and 150 mg soft capsules Summary of Product Characteristics. Boehringer Ingelheim.
Related products
Find out more information about OFEV® (nintedanib) below
OFEV® (nintedanib) 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.
PC-GB-106293
March 2022