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Act now against progressive pulmonary fibrosis

You have a crucial role in promptly recognizing and referring patients with suspected interstitial lung disease (ILD) to a lung specialist,1 as it can be progressive and deadly2

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Progressive pulmonary fibrosis in ILDs can be deadly3–13

pulmonary fibrosis can be deadly

Fibrotic ILD can be deadly2 – you have a crucial role in timely referral of suspected ILD patients to a lung specialist1

Dr. Nazia Chaudhuri outlines the physical and emotional impacts of the progressive fibrosing phenotype on patients and how it can develop in ILDs regardless of their underlying trigger

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My name’s Nazia Chaudhuri, I’m a chest physician and the clinical lead of the Interstitial Lung Disease unit at the Manchester University NHS Foundation trust. I’m also the Chief Investigator of the progressive fibrosing interstitial lung disease clinical trial.

What I thought I’d do today first, before I talk to you about the trial is just remind everybody what fibrosis means to a patients and what impact it has on our patients. One of the difficult things I find as a doctor when I have a patient in front of me with fibrosing lung disease, is to actually explain to them that it is an incurable disease, and it often is progressive.

Our patients will develop progressive breathlessness and cough, as well as anxiety and depression and at the moment, there’s very little evidence-based treatments for progressive interstitial lung disease bar idiopathic pulmonary fibrosis.

There are a number of fibrotic lung diseases that look like and behave like IPF. Like IPF, they have a progressive decline and there’s no established treatments to try and halt that decline. Even though the initial trigger may be different, it’s likely that the pathogenetic mechanisms and the end stage fibrosis is the same. These include patients with connective tissue diseases like rheumatoid arthritis that have similar decline and prognosis as idiopathic pulmonary fibrosis. Other conditions include chronic hypersensitivity pneumonitis, sarcoidosis and asbestosis. These conditions can all have progressive declines in their lung function and can have a major impact on patients with symptoms of breathlessness, cough and can ultimately reduce survival.

Fibrotic ILD patient’s independence and quality of life decline with progressive irreversible lung damage14–17 – you have an important role in the early recognition and timely referral of patients with suspected ILD to a lung specialist1

Abbreviations

cHP, chronic hypersensitivity pneumonitis; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc-ILD, systemic sclerosis-associated interstitial lung disease; uILD, unclassifiable interstitial lung disease.

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  12. Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax. 2018;73(4):391–392.
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