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Facts About ILD

The cause of interstitial lung disease is often not known.  Potential causes include: 

  • Certain infections
  • Radiation therapy
  • Certain medications
  • Occupations and exposures such as silica or asbestos, bird/feathers
  • Diseases such as rheumatoid arthritis, scleroderma, mixed connective tissue disease, and systemic lupus erythematosus. 
  • The most common cause of pulmonary fibrosis is idiopathic pulmonary fibrosis (pulmonary fibrosis of unknown cause).

Symptoms of interstitial lung diseases most commonly include:

  • shortness of breath, especially with exercise
  • dry cough that does not produce phlegm
  • fatigue and weakness
  • loss of appetite or weight
  • discomfort in chest
  • muscle or joint pains
  • clubbing of the fingers
  • Swelling in the legs

Evaluation and diagnosis of interstitial lung disease requires several types of testing:

  • Chest CT scans are performed to help determine the type and extent of lung disease as well as help guide a lung biopsy.
  • Blood tests are performed to diagnose autoimmune diseases such as rheumatoid arthritis, scleroderma, lupus.
  • Bronchoscopy with bronchoalveolar lavage and transbronchial biopsy. Bronchoalveolar lavage is a technique where washings from the lung are analyzed in the laboratory for the type of cells and degree of inflammation present, evidence of infection and for cancer cells.  Often a transbronchial biopsy is performed through the bronchoscope and yields small pieces of lung, which can be helpful in determining the cause of fibrosis.
  • Surgical lung biopsy is often required to make the diagnosis.
  • Pulmonary function studies and exercise tests are performed to determine how well the lungs are functioning and are repeated periodically to determine whether the lungs are responding to treatment. This consists of spirometry (to measure lung flow rates), lung volumes, diffusing capacity (to measure how well oxygen and other gases get from the air you breath into your bloodstream), and a 6 minute walk test (to measure your oxygen level when exercising and to measure your exercise endurance).
  • Often lung disease can affect the heart and heart tests such as an ultrasound are also required.

The treatment varies depending on the type of interstitial lung disease.

  • In some forms of interstitial lung diseases, anti-inflammatory drugs such as corticosteroids (prednisone) are used.
  • Often other medications are used to suppress the immune system and prevent or slow further lung scarring.  These medications include azathioprine or cyclophosphamide. These medications are usually given a trial of three to six months and if there is no benefit, then they are discontinued.
  • Other therapies include home oxygen therapy, smoking cessation, pulmonary rehabilitation and in some severe cases, lung transplantation is required.

For more information on pulmonary fibrosis, you can visit the following organizations and their patient education material:

Coalition for pulmonary fibrosis

Pulmonary Fibrosis Foundation