Pulmonary Fibrosis

Pulmonary fibrosis, including idiopathic pulmonary fibrosis, is a condition that occurs when the lung tissue becomes scarred. The stiff, thickened tissue makes it difficult for the lungs to work properly. As the condition worsens, it is more difficult for the lungs to transfer oxygen into the bloodstream, affecting the body’s ability to function.

Currently, the lung damage caused by pulmonary fibrosis can't be repaired, but medications and therapies can sometimes help ease symptoms, slow the rate of loss of lung function and improve quality of life. For some people, a lung transplant might be appropriate.

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Symptoms

The type and severity of pulmonary fibrosis symptoms vary, with some people becoming ill very quickly, while others have more moderate symptoms that worsen over months or years. 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 termed 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 tuberculosis, pneumonia, 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.

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Diagnosis

Diagnosis of pulmonary fibrosis usually begins with a physical exam, where a medical history is taken, including history of smoking, occupational exposure to materials that could irritate your lungs, history of drug use and other medical conditions. Other diagnostics include imaging tests, such as chest x-rays and CT scans, 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.  

Sometimes a bronchoalveolar lavage is performed during an examination of the lung known as a bronchoscopy. A bronchoalveolar lavage involves the removal and examination of cells from the lung. Lung biopsy, the removal of a lung tissue sample for study, may also be performed during bronchoscopy or as a surgical procedure.

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Treatments

There is no cure for idiopathic 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 drugs may help prevent further scarring and increase survival time in patients where the fibrosis is related to an autoimmune disease. They don't work for every one battling pulmonary fibrosis, however.

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.

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.

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