What is pulmonary fibrosis (scarring of the lungs)? In this episode I provide a thorough overview and highlight the difference between progressive and non-progressive lung fibrosis, causes, getting a clear diagnosis, follow-up over time and treatment.
Causes of pulmonary fibrosis may include: severe lung infections, such as tuberculosis (TB); systemic/autoimmune diseases; environmental factors, such as dusts, hypersensitivity to various things - bird proteins, mold, humidifiers, farm workers, compost, saw dust - literally anything; undetermined causes (idiopathic pulmonary fibrosis - IPF).
The diagnosis is usually made using a combination of tests, which may include: imaging - HRCT, respiratory tests (lung function tests), blood tests, bronchoscopy, lung biopsy. The results are then typically brought to a multidisciplinary discussion (MDT) where several specialists make the final diagnosis.
Treatment involves many components - it is not just medication. If the fibrosis is extensive or there is a risk of progression, it is extremely important to have regular check-ups with a doctor in order to carefully observe the fibrosis over time. This is usually done by repeating lung function and imaging over time, as well as monitoring symptoms.
Sometimes no treatment for fibrosis may be recommended or there may not be effective therapy available, and in such cases symptomatic treatment may sometimes be given.
Eliminating the cause is extremely important. Stopping smoking usually helps, as well as paying attention to causes from the environment, in case there is a component of hypersensitivity (birds, moldy hay, animals, agriculture etc.).
Medication usually involves anti-inflammatory or antifibrotic treatment, which is chosen depending on the cause of the lung fibrosis. Anti-inflammatory agents may include corticosteroids (e.g. prednisone) or immunosuppressants (methotrexate, mycophenolate). Antifibrotic drugs which are currently approved include nintedanib (Ofev) and pirfenidone (Esbriet). These are both indicated for idiopathic pulmonary fibrosis (IPF), while nintedanib (Ofev) can sometimes be used in other forms of progressive fibrosis (which meet certain criteria).
Oxygen therapy is also very important as a form of treatment, to correct low levels in the blood and ensure that other organs such as the heart are operating efficiently.
Pulmonary rehabilitation by maintaining physical exercise is extremely important. Keeping fit to the extent possible will ensure that the body will cope with the reduction in lung function better.
Treatment of symptoms may involved having medication for chronic dry cough or shortness of breath. Treatment of associated diseases which may contribute to these symptoms is also essential (i.e. it is not always only the fibrosis causing the symptoms). Psychological counseling and cognitive-behavioral therapy can help with anxiety and panic attacks which may occur due to sudden episodes of breathlessness and changes in lifestyle.
Participation in clinical trials helps drive the science of pulmonary fibrosis forward and may offer early access to innovative therapies. Taking part in research also means that the condition is observed more carefully, with regular lung function and consultations.
I do encourage patients to join local support groups and patient associations in order to meet others coping with pulmonary fibrosis and get updates from a patient perspective. Some of these groups can be found by asking at the local hospital or on social media (i.e Facebook).
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