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CONDITIONS

Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis (PF) is a lung disease characterized by the gradual scarring of lung tissue. This scarring, or fibrosis, makes the lungs stiff and decreases their ability to expand, leading to difficulty breathing. Breathing in harmful chemicals is one possible cause of pulmonary fibrosis. Specific illnesses, medications, or genetics can also cause it. Most often, the cause remains unknown. This is termed idiopathic pulmonary fibrosis (IPF).  

How does pulmonary fibrosis affect breathing?

The lungs contain tiny air sacs called alveoli. When lungs are healthy, oxygen easily passes through the walls of the air sacs and into the blood. However, with pulmonary fibrosis, the tissue around the air sacs becomes stiff and thick. This can lead to permanent lung scarring, making the walls even thicker. The thickened walls of the air sacs make it harder for oxygen to pass into the bloodstream.

What causes pulmonary fibrosis?

Known causes include:

  • Exposure to asbestos and inorganic dust at work, organic compounds like mold
  • Certain medications e.g. amiodarone, nitrofurantoin
  • Autoimmune diseases such as scleroderma or rheumatoid arthritis, as well as chemotherapy or radiation therapy.
  • Smoking is a risk factor for many types of PF.

 

Despite thorough testing, specialists often cannot identify a cause for a person’s PF. In such cases, it is labelled as idiopathic. The most common idiopathic form of PF is a specific disease called idiopathic pulmonary fibrosis (IPF).

What are the risk factors for pulmonary fibrosis?

Risk factors for pulmonary fibrosis include:

  • Advanced age: Pulmonary fibrosis usually develops in individuals aged 50 to 70.
  • Male gender: Men are more likely than women to have pulmonary fibrosis.
  • Smoking: Cigarette smoking increases the risk of pulmonary fibrosis.
  • Occupational exposure: Regular inhalation of chemicals or hazardous substances, common in farming, hairdressing, stone cutting/polishing, and metal working, can damage the lungs.
  • Coexisting medical conditions: Certain autoimmune conditions like rheumatoid arthritis, scleroderma may contribute to pulmonary fibrosis.
  • Other factors: Exposure to radiation, such as during cancer treatment, as well as certain medications like chemotherapy or specific heart medications, can also damage lung tissue.

What are the signs and symptoms?

People with pulmonary fibrosis often experience these symptoms:

  • Shortness of breath that worsens over time. Initially, breathing may be more difficult during activity. Eventually, there could be shortness of breath even at rest.
  • Persistent dry cough. This might involve uncontrollable coughing bouts that do not improve.
  • Aching joints and muscles.
  • Fatigue or weakness even during light activity
  • Gradual, unintentional weight loss.

As the disease progresses, some people may experience:

  • Finger and toe clubbing: Tips of fingers and toes become enlarged, and the nails become curved (a sign that there is not enough oxygen in the blood)
  • Cyanosis: Bluish fingertips, ear lobes, and lips

How is pulmonary fibrosis diagnosed?

Pulmonary fibrosis is diagnosed with the help of:                       

  • Medical History and Physical Examination: The doctor will review medical history including symptoms, and conduct a physical examination; there may be an abnormal sound (crackling) on chest auscultation.
  • Imaging Tests: Imaging tests like chest X-rays or computed tomography (CT) scans provide detailed images of the lungs, helping to identify scarring patterns and assess the extent of lung damage.
  • Pulmonary Function Tests (PFTs): These tests measure lung function, including how much air lungs can hold and how effectively an individual can breathe in and out. PFTs can help detect abnormalities in lung function associated with pulmonary fibrosis.
  • 6-Minute Walk Test: A six-minute walk test is used to assess the exercise capacity. This test assesses how the body utilizes oxygen during exercise and determines activity tolerance. It is common for people with PF to experience low oxygen levels during the test and to walk a shorter distance as the disease advances.
  • Blood Tests: Blood tests may be performed to check for signs of inflammation, autoimmune conditions, or other underlying diseases that could contribute to pulmonary fibrosis.
  • Bronchoscopy: In some cases, a bronchoscopy may be done to examine the airways and collect samples (biopsies) of lung tissue for further analysis.
  • Biopsy: A lung biopsy involves removing a small sample of lung tissue for examination under a microscope. This can help confirm the presence of pulmonary fibrosis and determine its severity.
  • Other Tests: Additional tests such as genetic testing or assessments for specific autoimmune markers may be conducted depending on individual circumstances.

How is pulmonary fibrosis managed?

The primary aim of most pulmonary fibrosis treatments is to alleviate symptoms and enhance quality of life.

  • Medications prescribed which may slow down the lung scarring and help preserve lung function.
  • Pulmonary rehabilitation includes exercise training and breathing exercises training to help an individual to enhance strength and overall well-being.
  • Adopting healthy lifestyle changes such as quitting smoking, exercising regularly, and maintaining a nutritious diet.
  • Oxygen therapy to facilitate breathing and exercise.
  • Lung transplant surgery in advanced cases.
  • Seeking counselling and support to manage stress and anxiety.

Managing pulmonary fibrosis may also involve regular checkups with a doctor (lung specialist). These doctor visits can guide avoiding exacerbating symptoms, knowing when to seek medical assistance, and enhancing quality of life.

In conclusion, living with pulmonary fibrosis can present challenges, but there are various treatments and strategies available to manage symptoms and improve quality of life. From oxygen therapy and medication to pulmonary rehabilitation and healthy lifestyle changes, individuals can take proactive steps to slow the progression of the disease and maintain their well-being.

References

  1. What is pulmonary fibrosis? Available at https://www.nhlbi.nih.gov/sites/default/files/publications/WhatIsPulmonaryFibrosis.pdf. Accessed on 25th April 2024
  2. Pulmonary Fibrosis Foundation. Available from https://www.pulmonaryfibrosis.org/docs/default-source/programs/educational-materials/pf-information-guides/pf-info-guide.pdf?sfvrsn=129c58c8_29 Accessed on 25th April 2024
  3. American Lung Association. Introduction to Pulmonary Fibrosis. Available from: https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/introduction Accessed on 25th April 2024
  4. Cleveland Clinic. Pulmonary Fibrosis. Available from: https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis Accessed on 25th April 2024
  5. Canadian Pulmonary Fibrosis Foundation. Pulmonary fibrosis patient guide. Available from: https://cpff.ca/wp-content/uploads/2023/03/03_01_2023_CPFF-Patient-Guide-March-1.pdf Accessed on 25th April 2024