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Pulmonary Fibrosis

 

What is pulmonary fibrosis?

Pulmonary fibrosis (PF) is a rare condition in which lung tissues become scarred, thick and stiff. Specifically, the walls of the air sacs in your lungs can become thickened resulting in a chronic cough, shortness of breath, feeling tired and low oxygen levels in your body.

What causes pulmonary fibrosis?

In most cases, the exact cause for PF is not known. When this occurs, the condition is called idiopathic pulmonary fibrosis. Some experts believe a genetic component may exist because PF often runs in families. Other causes can include:

  • Exposure to inhaled chemicals, such as asbestos, grain dust and silica.
  • Exposure to bird droppings.
  • Radiation therapy to the chest.
  • Certain medications, such as chemotherapy, heart medications and some antibiotics.
  • Those with certain medical conditions, such as lupus, scleroderma, sarcoidosis, or rheumatoid arthritis.
  • Those who smoke or have formerly smoked.

PF is most commonly found in adults, although it can occur in children and infants. Factors that can increase your risk of PF include:

  • Currently smoking or previously smoking.
  • Individuals with emphysema.
  • Certain work conditions, such as mining, farming or construction.
  • Family history of PH.

What are symptoms of pulmonary fibrosis?

The progression and severity of symptoms can vary from person to person. Some people can become ill very quickly, others have symptoms that progress slowly, over months or years. The most common symptoms of pulmonary fibrosis may include:

  • Shortness of breath.
  • Dry cough.
  • Extreme tiredness.
  • Weight loss that’s not intended.
  • Aching muscles and joints.
  • Widening and rounding of the tips of the fingers or toes.

In people with PF, shortness of breath can suddenly get worse over a few weeks or days, called acute exacerbation. This may be caused by another condition or illness, such as a lung infection. Acute exacerbations can be life-threatening, so contact your provider right away if you notice suddenly worse symptoms.

How is pulmonary fibrosis diagnosed?

To diagnose pulmonary fibrosis, your provider will perform a physical examination and review your medical history. You will likely be asked about any continuous or repeated contact with dusts, gases, chemicals or similar inhalants. Your provider will listen closely to your lungs to determine if there is a crackling sound that is often seen with PF. Other kinds of tests may also be needed to help your provider diagnose your condition.

Imaging tests may be needed to show the extend of the damage to your lungs, including:

  • Chest X-ray – may show scar tissue that is common with PF.
  • CT scan – may show how much lung damage has occurred.
  • Echocardiogram – uses soundwaves to look at the heart and can help determine how the heart is working.

Pulmonary function tests will help your provider see how well your lungs are working. These may include:

  • Spirometry – involves breathing out quickly and forcefully through a tube connected to a machine. This test measures the amount of air you can inhale and exhale and whether your lungs deliver enough oxygen to your blood.
  • Lung volume test – measures the amount of air the lungs hold at different times when breathing in and out.
  • Lung diffusion test – shows how well the body moves oxygen and carbon dioxide between the lungs and the blood.
  • Pulse oximetry – uses a small device placed on the finger to determine how much oxygen is in the blood.
  • Exercise stress test – involves light exercise on a treadmill or stationary bike to monitor heart and lung function during activity.
  • Arterial blood gas test – involves taking a sample of blood from an artery in the wrist to measure oxygen and carbon dioxide levels.

If imaging tests and pulmonary function tests do not help to find the cause of your condition, a tissue biopsy may be needed. A biopsy is a small amount of tissue that is taken from the affected area to be examined in a laboratory to diagnose a condition. In this case, to diagnose pulmonary fibrosis or rule out other conditions. Methods of obtaining a biopsy include:

  • Thoracotomy – also known as a surgical biopsy. This method is invasive and has potential complications. During a thoracotomy, your surgeon removes a lung tissue sample through a cut that opens between the ribs.
  • Bronchoscopy – a small, flexible tube is passed through the mouth or nose into the lung to remove a very small tissue sample. Sometimes, the tissue sample size is too small and a surgical biopsy is needed.

How is pulmonary fibrosis treated?

Treatment for PF will look different for each person, depending on the severity of your condition. The lung scarring and thickening that occurs in PF cannot be repaired, and there is no current treatment that prevents the disease from worsening overtime. Treatments aim to improve your symptoms, improve quality of life or slow down the progression of your PF. Common treatments for PF include:

  • Medication – certain medications can help control symptoms and slow the worsening of PF.
  • Oxygen therapy – extra oxygen cannot slow lung damage, but it can make breathing and exercise better, improve your sleep and prevent or lessen complications from low blood oxygen levels. If you require oxygen all the time, your provider may recommend a portable oxygen concentrator.
  • Pulmonary rehabilitation – can help you manage your symptoms and improve your ability to do daily tasks. This treatment option focuses on breathing techniques, nutritional counseling, emotional support and improving your stamina for physical exercise.

For people with severe PF, a lung transplant may be an option. A lung transplant can improve your quality of life and help you to live a longer, healthier life, but it also carries several risk factors and potential complications, such as rejection or infection. People who receive a lung transplant require medication for the duration of their life. Your provider will talk with you to determine if this is the right treatment plan for you.

What are risk factors of pulmonary fibrosis?

If left untreated, pulmonary fibrosis can have potentially life-threatening complications, including:

  • High blood pressure in the lungs – also called pulmonary hypertension. Stiff and thick arteries block the blood flow through the lungs which raises the pressure inside the pulmonary arteries and lower right heart chamber.
  • Right-sided heart failure – occurs when the right side of your heart has to pump harder to move blood through blocked pulmonary arteries.
  • Respiratory failure – often the last stage of lung disease and occurs when blood oxygen levels fall very low.
  • Lung cancer – untreated PF increases your risk of developing lung cancer.
  • Other serious medical conditions – such as blood clots, collapsed lung or lung infections.

If you notice your symptoms suddenly get worse, contact your healthcare provider right away.