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
Progressive pulmonary fibrosis in ILDs can be deadly3–13
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.
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.
- Zibrak JD, Price D. Interstitial lung disease: raising the index of suspicion in primary care. NPJ Prim Care Respir Med. 2014;24:14054.
- Wijsenbeek MS, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35(11):2015–2024.
- Ryerson CJ, Urbania TH, Richeldi L, et al. Prevalence and prognosis of unclassifiable interstitial lung disease. Eur Respir J. 2013;42:750–757.
- Hyldgaard C, Bendstrup E, Wells AU, Hilberg O. Unclassifiable interstitial lung diseases: Clinical characteristics and survival. Respirology. 2017;22(3):494–500.
- Wells AU, Cullinan P, Hansell DM, et al. Fibrosing alveolitis associated with systemic sclerosis has a better prognosis than lone cryptogenic fibrosing alveolitis.
Am J Respir Crit Med.1994;149(6):1583–1590.
- Mapel DW, Hunt WC, Utton R, et al. Coultas DB. Idiopathic pulmonary fibrosis: survival in population based and hospital-based cohorts. Thorax. 1998;53(6):469–476.
- Hubbard R, Johnston I, Britton J. Survival in patients with cryptogenic fibrosing alveolitis: a population-based cohort study. Chest. 1998;113(2):396–400.
- Goh NS, Desai SR, Veeraraghavan S, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med.
- Moore OA, Goh N, Corte T, et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease. Rheumatology (Oxford). 2013;52(1):155–160.
- Kim Reference EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease. Eur Respir J. 2010;35(6):1322–1328.
- Bongartz T, Nannini C, Medina-Velasquez YF, et al. Incidence and mortality of interstitial lung disease in rheumatoid arthritis: a population-based study. Arthritis Rheum. 2010;62(6):1583–1591.
- Gimenez A, Storrer K, Kuranishi L, et al. Change in FVC and survival in chronic fibrotic hypersensitivity pneumonitis. Thorax. 2018;73(4):391–392.
- Vourlekis JS, Schwarz MI, Cherniack RM, et al. The effect of pulmonary fibrosis on survival in patients with hypersensitivity pneumonitis. Am J Med.
- Swigris JJ, Brown KK, Abdulqawi R, et al. Patients’ perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):pii:180075.
- Wuyts WA, Papiris S, Manali E, et al. The burden of progressive fibrosing interstitial lung disease: a DELPHI approach. Adv Ther. 2020;37(7):3246–3264.
- Wells AU, Brown KK, Flaherty KR, et al. What’s in a name? That which we call IPF, by any other name would act the same. Eur Respir J. 2018;51(5):1800692.
- 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.