Pulmonary Fibrosis: What is it, Symptoms, Causes and Treatment

Pulmonary Fibrosis: What is it, Symptoms, Causes and Treatment

The word “pulmonary” means lung and the word “fibrosis” means scar tissue. Pulmonary fibrosis (PF) therefore refers to a medical condition where there is scarring in the tissue of the lungs. With continued scarring, the architecture of the lung stiffens, making it less efficient at getting oxygen into the bloodstream and it becomes increasingly more difficult to breathe as expanding the lungs becomes harder to achieve. As it takes more energy to breathe and shortness of breath may develop with activities that were previously easier to do, such as walking and exercising. Most people with PF develop symptoms between the ages of 50 and 70 years. According to the American Lung Association, the main symptoms of PF are:

  • Shortness of breath, particularly during exercise
  • Dry, hacking cough
  • Fast, shallow breathing
  • Gradual unintended weight loss
  • Tiredness
  • Aching joints and muscles
  • Clubbing (widening and rounding) of the tips of the fingers or toes

Diagnosis based on these symptoms alone is not possible though, as pulmonary fibrosis can look a lot like other lung diseases and therefore make diagnosis challenging. Healthcare providers may order additional tests to diagnose pulmonary fibrosis such as blood tests, imaging tests, pulmonary function tests, and biopsies etc.

Causes of Pulmonary Fibrosis

There are several known causes of pulmonary fibrosis as well as unknown causes, which are referred to as idiopathic. Of the known causes there are five main categories according to the Pulmonary Fibrosis Foundation and these are medications, radiation, environmental exposures, autoimmune diseases, and occupational exposures.

How Is Pulmonary Fibrosis Treated?

Once scarring of tissue occurs in the lungs it cannot be reversed. Because of this, there is currently no cure for pulmonary fibrosis. Most pulmonary fibrosis treatments are aimed at slowing the course of the disease, relieving symptoms, and helping patients stay active and healthy to improve their quality of life. As part of ongoing treatment, spirometry, and the ability to instantly share lung function data with the doctor play an important role. For most people with PF, monitoring their FVC values can help their doctor decide whether their disease is stable or progressing and whether their disease is mild, moderate, or severe. It is therefore important for PF patients to regularly test their lung conditions and to discuss their results with their doctor. With home spirometers, patients can perform their spirometry tests at home with clinical accuracy and share their results with healthcare providers.


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