Pulmonary Fibrosis

Pulmonary fibrosis is a condition that occurs when the lung tissue becomes scarred. This damaged tissue becomes thicker and stiffer, which causes the lungs to work less efficiently. As the condition worsens, it is more difficult for the lungs to transfer oxygen into the bloodstream, affecting the body’s ability to function.

There is currently no treatment that can repair the damaged tissue in the lungs, but medications and therapies can help many patients manage symptoms, slow the loss of lung function and improve their quality of life. Because symptoms of the condition become worse over time (progressive), pulmonary fibrosis can be fatal.


Symptoms of pulmonary fibrosis vary from one patient to the next. Symptoms most commonly develop over time and progressively become worse. Some patients may notice symptoms that worsen very quickly, while others may notice less severe symptoms that progress over a much longer period of time. Symptoms also are at least partially dependent on the cause of the fibrosis.

The symptoms of pulmonary fibrosis include:

  • 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)

Causes and Risk Factors

Pulmonary fibrosis is associated with many conditions, but in most cases, the cause is never found. When this is the case, the condition is known as idiopathic pulmonary fibrosis. There are several theories being researched as to what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. There are also types of idiopathic pulmonary fibrosis that appear to be hereditary, so there is likely a genetic component to the disorder.

Causes known to be associated with pulmonary fibrosis:

  • Occupational and environmental factors, including long-term exposure to silica dust, asbestos fibers, grain dust, bird and animal droppings.
  • Radiation treatments, after which damage can appear months or years after initial treatment. Severity of damage depends on how much of the lung was exposed to radiation, total amount of radiation administered, whether chemotherapy also was used, presence of underlying lung disease.
  • Medications, including chemotherapy drugs such as methotrexate and cyclophosphamide, some drugs used to treat irregular heartbeats such as amiodarone or propranolol, some antibiotics such as nitrofurantoin and sulfasalazine.
  • Medical conditions, including systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis or scleroderma.

Although the condition has been found in children and infants, it is much more likely to affect middle-aged and older adults, particularly those with a history of smoking. People who work in mining, farming or other occupations that regularly expose them to pollutants are also more susceptible to pulmonary fibrosis.


Diagnosis of pulmonary fibrosis usually begins with a physical exam and a review of the patient's medical history and symptoms. During the this time, the physician will pay special attention to the patient's history of smoking, occupational exposure to materials that could irritate the lungs, history of drug use and other medical conditions.

Imaging diagnostic tests may include chest x-rays and CT scans, which can provide a better understanding of patient’s condition by showing an image of what is going on inside the lungs. These diagnostic images can show if fluid has begun to build up in the chest or lungs as well as determine if the lungs are fully inflating.

Other tests may include blood oxygen tests as well as lung function and exercise testing to find out how much damage has been done and how well the lungs are operating. The medical team may also examine the airways using a bronchoscopy. The bronchoscope is a flexible, hollow tube that is inserted through the mouth or nose and into the bronchi (windpipes). The procedure may be performed under light sedation or a general anesthetic. A tissue sample may be taken during the bronchoscopy and later examined under a microscope.

If fluid has begun to build up in the chest or lungs, the physician may take a sample of the fluid to test it in order to rule out cancer.


There is no cure for pulmonary fibrosis. Current treatments are aimed at preventing more damage and relieving symptoms. There is no known way to repair scarring that has already occurred.

Current treatments include anti-inflammatory medication, such as corticosteroids, or other medications that suppress the immune system. These medications may help prevent further scarring and increase survival time in patients where the fibrosis is related to an autoimmune disease. However, they don't work for everyone experiencing pulmonary fibrosis.

Oxygen therapy may be prescribed if the amount of oxygen in the blood is low, which may help with shortness of breath and prolong the patient's ability to stay active. Many doctors will recommend pulmonary rehabilitation, which provides information and counseling to patients to help them manage the condition.

Gastroesophageal reflux may contribute to the progression of pulmonary fibrosis and should be evaluated and treated when present.

Patients with pulmonary fibrosis may need to receive flu and pneumonia vaccinations to reduce their chances of infection.

For some, a lung transplant may be the best option, particularly those younger than 65 with no other medical problems who aren't responding to medications.

Importantly, there are clinical trials ongoing for patients with idiopathic pulmonary fibrosis. Several of these trials are in Phase III and hopefully, new therapies will be approved in the United States within the next year or two. There are also Phase II trials that Cedars-Sinai will be participating in.

The knowledgeable and highly trained staff at the Women's Guild Lung Institute work as a multidisciplinary team to determine the best treatment option for each patient.