Pulmonary Fibrosis - Definition, Causes, and Prognosis

Causes, Symptoms, and Treatment of Pulmonary Fibrosis

What is pulmonary fibrosis?. istockphoto.com

What is pulmonary fibrosis and what are the causes?

Definition: Pulmonary Fibrosis

Pulmonary fibrosis is a scarring of the lungs which may be due to many causes. It is categorized as part of a group of lung diseases known as interstitial lung disease. People with pulmonary fibrosis typically have progressive shortness of breath. The treatment and prognosis of pulmonary fibrosis can vary depending upon the cause, but in general, there is no cure for pulmonary fibrosis.

On a microscopic scale, the walls of the alveoli (the tiny air sacs in the lungs) become scarred. This prevents the alveoli from expanding and contracting well during breathing.


There are many causes of pulmonary fibrosis, some of these include:

  • Idiopathic pulmonary fibrosis - This is when no definite cause can be found for fibrosis in the lungs. To learn more about IPF check out this 3 part series on idiopathic pulmonary fibrosis including symptoms and treatment.  In addition to causing symptoms on its own, IPF is a risk factor for developing lung cancer.
  • Infections, especially viral infections.
  • Exposure to toxic substances (such as asbestos and silica dust).
  • Allergies.
  • Some medications such as the chemotherapy drug Cytoxan, and the bladder infection drug Macrodantin (nitrofurantoin.)
  • Pneumoconioses such as black lung disease.
  • Radiation therapy to the chest for lung cancer, breast cancer, or lymphomas. Lung fibrosis usually begins with radiation pneumonitis - and inflammation of the lungs due to radiation therapy. If radiation pneumonitis is not diagnosed, or if it is inadequately treated, permanent radiation fibrosis can be a result. Researchers are looking at ways to help prevent this side effect of radiation therapy for cancer.  Pulmonary fibrosis may develop months to years after radiation treatments are completed.
  • Genetics - It appears that sometimes pulmonary fibrosis runs in families suggesting a genetic element.
  • Gastrointestinal reflux disease (GERD) - Longstanding acid reflux may be a risk factor for lung fibrosis.


Symptoms of pulmonary fibrosis may include:

  • Shortness of breath, often beginning with exercise and then progressing until someone is short of breath at rest. This is the most common symptom of pulmonary fibrosis.
  • A dry cough.
  • Chest pain.
  • Palpitations.
  • Weight loss.
  • Fatigue.
  • Muscle and joint aches.
  • Clubbing - This is a condition in which the end of the fingers take on the look of an inverted spoon.


The diagnosis of pulmonary fibrosis may include a combination of the tests below, as well as tests to rule out some of the causes of the disease.


The treatment of pulmonary fibrosis will depend on the cause. Recently, treatment has become available specifically for people with idiopathic pulmonary fibrosis.

Since pulmonary fibrosis is by definition permanent, treatment is aimed at preventing worsening of the disease and to help people cope with the symptoms of the disease. Medications may help prevent further inflammation, and pulmonary rehabilitation, medications, and oxygen therapy when severe, may help people lead an active life despite suffering from pulmonary fibrosis. For some people, a lung transplant is a potential option, and currently, pulmonary fibrosis is the leading reason for lung transplants in the United States.


Since the lungs become less elastic, you may picture it being more difficult for blood to pass through the lungs - which in turn can result in the blood pressure being high in these blood vessels, and the part of the heart working to pass blood through the lungs having to work harder. Some complications may include:

  • Pulmonary hypertension - Increased blood pressure in the pulmonary blood vessels
  • Cor pulmonale - Heart failure involving the right side of the heart (most of the time heart failure means left heart failure, with the heart having difficulty pumping blood to the rest of the body.)
  • Heart disease.
  • Lung cancer - Pulmonary fibrosis is associated with an increased risk of developing lung cancer.
  • Blood clots in the legs which can travel to the lungs (pulmonary emboli.)


The prognosis of pulmonary fibrosis also depends on the particular cause. In general, the life expectancy for someone with pulmonary fibrosis is between 3 and 5 years, but this can vary significantly for different causes and between different people.


American Lung Association. Pulmonary Fibrosis. Accessed 06/09/16. http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-fibrosis/

National Library of Medicine. MedlinePlus. Pulmonary Fibrosis. Updated 07/13/16. https://medlineplus.gov/pulmonaryfibrosis.html

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