The term ‘pulmonary fibrosis’ (PF) is often used to describe a group of interstitial lung diseases (ILD), a range of rare conditions that causes inflammation and scarring in the lungs. As the lungs scar and stiffen, it increasingly becomes difficult to breathe due to restriction and as not enough oxygen is able to enter the bloodstream.
There are over 200 different types of ILD/PF. Some people may already have features of associated conditions such as autoimmune diseases like rheumatoid arthritis, scleroderma or Sjogren’s syndrome. Environmental factors, genetic links, sinus disease and reflux aspiration can all play a role.
Idiopathic Pulmonary Fibrosis (IPF) is the most common type of ILD/PF. The word “idiopathic” means the underlying cause of the disease is unknown. Idiopathic pulmonary fibrosis is a chronic, progressive and fatal lung disease resulting in severe shortness of breath and impaired quality of life as the lung function declines. Anti-fibrotic medicines can slow the rate of decline but a referral to the lung transplant service for an assessment may also be required.
Specialist Screening Healthcare Ireland recognises the importance of specialist pulmonary radiologists with an interest in interstitial lung disease/pulmonary fibrosis reporting on CT scans. Diagnosis of subtypes can be helped by accurate interpretation of CT images combined with a multidisciplinary meeting (MDM) consensus opinion. LungCheck will arrange referral to treatment specialists, including interstitial lung disease/pulmonary fibrosis respiratory experts, rheumatologists and if required, lung transplant physicians.
The symptoms of interstitial lung diseases/pulmonary fibrosis can be similar to symptoms of other lung diseases and therefore often goes misdiagnosed. The course of ILD/PF and the severity of symptoms can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.
Symptoms of pulmonary fibrosis usually have a gradual onset and may include:
Shortness of breath – at first only when physically active, but then progresses to when you are just resting.
Fast, shallow breathing.
A dry, hacking cough that does not go away. Sometimes clear sputum is being coughed up.
Fatigue and weakness.
Aching joints and muscles.
Gradual, unexplained weight loss.
Clubbing, or enlargement, of the ends of the fingers (or sometimes the toes) due to a build-up of tissue.
This disease is much more likely to affect middle-aged and older adults between the ages of 50 and 70 years of age.
Interstitial lung disease/pulmonary fibrosis is more common in men, but the number of cases in women is on the rise.
Far more smokers and former smokers develop ILD/PF than do people who have never smoked. Interstitial lung disease/pulmonary fibrosis can occur in patients with Emphysema/COPD. This is called combined Pulmonary Fibrosis Emphysema Syndrome (CPFE).
People exposed to the following elements at work have a greater likelihood of developing ILD/PF:
If you are exposed to long-term toxins and pollutants such as silica dust, asbestos fibres, hard metal dusts, coal dust, grain dust or allergens carried in the air such as bird and animal droppings, all known to damage your lungs, consider a LungCheck screen to out rule any early signs of ILD/PF.
LungCheck supports business owners and their employees by offering specialist diagnostics to exclude lung disease linked to occupational exposures.
Certain drugs can have the adverse effect of scarring your lungs causing damage. Having radiation treatments to your chest or using certain chemotherapy, heart, antibiotics, anti-inflammatory and recreational drugs can increase your risk of interstitial lung disease/pulmonary fibrosis.
Some types of interstitial lung disease/pulmonary fibrosis run in families however this is very rare. When two or more members within the same family have idiopathic pulmonary fibrosis (IPF) or any other form of idiopathic interstitial pneumonia (IIP), family history plays a key risk factor.
Researchers have noted a number of medical conditions including viruses such as Covid-19 that can trigger pulmonary fibrosis. There are many conditions that can cause pulmonary fibrosis with long-term scarring of the lungs such as:
The Irish Thoracic Society has established an Interstitial Lung Disease (ILD) registry, which is providing data on disease characteristics in Ireland with particular focus on pulmonary fibrosis. The ILD Annual Report published in 2018 from the Irish Thoracic Society provides preliminary data on trends in the diagnosis, management and treatment of Idiopathic Pulmonary Fibrosis in Ireland (IPF).
If clinically indicated, on completion of diagnostics Specialist Screening Healthcare Ireland’s LungCheck service offers referral with direct access to multidisciplinary meetings with referral pathways linked to interstitial lung disease specialists.
In selected patients, lung transplantation may be a treatment option, with optimal results in quality of life and survival. Currently, all forms of pulmonary fibrosis are the main reason for lung transplantation in Ireland.