Act now against progressive pulmonary fibrosis
Your prompt recognition of fibrotic ILD through HRCT is important, as early diagnosis can help reduce the burden faced by patients with an uncertain diagnosis1–4
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.
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.
Potential burden faced by patients prior to fibrotic ILD diagnosis:3
Management options are available for fibrotic ILDs, making early diagnosis imperative1,2
Misdiagnosis of ILD can lead to frequent physician visits, repeated diagnostic tests, and exposure to ineffective treatment1,3
*IPF is a well-known example of a fibrotic ILD that is always progressive.7
HRCT, high-resolution computed tomography; ILD, interstitial lung disease; IPF, idiopathic pulmonary fibrosis.
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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;12:537–539.
- Cosgrove GP, Bianchi P, Danese S, Lederer DJ. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med. 2018;18(1):9.
- Lamas DJ, Kawut SM, Bagiella E, Philip N, Arcasoy SM, Lederer DJ. Delayed access and survival in idiopathic pulmonary fibrosis: a cohort study. Am J Respir Crit Care Med. 2011;184(7):842–847.
- Ley B, Collard HR, King Jr TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–440.
- Schoenheit G, Becattelli I, Cohen AH. Living with idiopathic pulmonary fibrosis: an in depth qualitative survey of European patients. Chron Respir Dis. 2011;8(4):225–231.
- Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev. 2019;28:180100.