Idiopathic Pulmonary Fibrosis
What is IPF and how common is it?
Idiopathic pulmonary fibrosis (IPF) is a debilitating chronic, progressive lung inflammation (pneumonia) characterised by fibrosis and disruption of the normal lung architecture leading to increasing impairment of gas exchange and often to respiratory failure and death.
There is no single known cause of IPF. Rather, IPF is likely caused by a multifactorial interaction between genetic risk factors and environmental risk factors such as smoking and occupational dust inhalation.
Every year, between 3 to 18 persons per 100,000 persons are diagnosed with IPF in Europe and the United States (about 30,000 to 40,000 new cases per year in the United States). IPF is more common in men and older age; it is very rare below the age of 50 years and the median age-of-diagnosis is around 65 years.
What are the symptoms and consequences of IPF?
IPF is an irreversible, chronically progressive disease with a highly variable disease course. The average life expectancy at diagnosis is only 3-5 years. A chronic dry cough that is resistant to standard cough medications as well as increasing shortness of breath on minimal exertion are major symptoms of IPF with an enormous negative impact on affected persons’ quality of life. Other symptoms include fatigue, tiredness, loss of appetite, and loss of weight. Shorter survival is associated with older age, lower body mass index, severe symptoms and impairment, greater disease extent on imaging, and the co-existence of other lung diseases such as emphysema and cancer.
How is IPF diagnosed?
The diagnosis of IPF is made based on the identification of radiological and/or histological patterns of interstitial pneumonia in patients with symptoms such as shortness of breath and dry cough after exclusion of other known causes of interstitial lung disease. High-resolution CT scanning is a common imaging modality used for diagnosis and to track disease progression; lung biopsy is also sometimes used to confirm the diagnosis. IPF diagnosis is based on a complex diagnosis of exclusion; patients are usually assessed and treated by a multidisciplinary team comprising radiologists, physicians, pathologists, palliative care specialists and others.
How is IPF managed?
Treatment options are limited and there is no approved treatment for the indication of chronic cough in IPF. Two disease-modifying treatments – nintedanib and pirfenidone – are approved in most countries to slow the decline in lung function and possibly improve survival. Both are associated with side effects such as gastrointestinal and skin-related irritations and up to one in five patients discontinues treatment because of disease progression or side effects. Adjunctive symptomatic therapy is important and may include opiates to reduce anxiety and shortness of breath, pulmonary rehabilitation, education and support programmes, and supplemental oxygen therapy. Often, the symptoms are refractory to standard pharmacological treatments and persistent cough is a significant physical and emotional burden in affected persons. There is a large unmet clinical need for a safe, well-tolerated treatment that reduces IPF patients’ cough and improves their quality of life.
References
1. Richeldi et a. Lancet 2017;389:1941-52. 2. Mattoo et al. Cellular Mol Life Sci 2021;78:5527-42. 3. Mei et al. Frontier Pharmacol 2022;12:797292. 4. Rozenberg et al. J Pain Symptom Management 2020;59:1362-78.