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Management of Interstitial (ILD) and Fibrotic Lung Disease

How is ILD diagnosed?
What is the treatment for ILD?
What are some of the medications used to treat ILD?
What are some other therapies?

How is ILD diagnosed?

When the diagnosis of ILD is suspected, your doctor considers the following:

Your Medical History:

This includes a review of any symptoms suggestive of connective tissue diseases, a listing of all current and former medications, and a review of any environmental and occupational exposures to dusts, gases, chemicals, pets (in particular birds), air conditioners, and humidifiers.

A Complete Physical Examination:

Findings of crackling sounds in the lungs and changes in the skin, joints and fingernails can help direct further evaluation.

A High Resolution Chest Computed Tomography (CT) Scan:

Results are usually abnormal and can sometimes help determine the type of ILD present.

Pulmonary Function Tests:

These breathing test results are usually abnormal in ILD. Your lung function may be checked before and after an inhaled bronchodilator treatment.

Arterial Blood Gas:

This test measures the amount of oxygen and carbon dioxide in your blood. The results may be normal or show a reduced oxygen level.

Exercise Testing:

This test may show a reduction in your oxygen level during to exercise.

Bronchoscopy With Bronchoalveolar Lavage or Biopsy:

Bronchoscopy may be done to check for inflammatory cells in your lungs or sample small pieces of the lung to look for evidence of a specific ILD. Bronchoscopy involves inserting a tube through the nose into your trachea (windpipe) to see the airways. In bronchoalveolar lavage, a small amount of sterile saline is placed in one area of your lung and then withdrawn. This fluid contains cells that will be analyzed under the microscope. Small biopsy tools may be used through the bronchoscope if indicated.

Surgical Lung Biopsy:

In many cases of ILD, a surgical lung biopsy is needed to get enough lung tissue to make a specific diagnosis. This is often performed with the use of a thoracoscope which allows the surgeon to biopsy multiple areas of one lung through a few very small incisions.

What is the treatment for ILD?

The goals of treatment are:

  • To decrease inflammation and prevent further lung scarring.
  • To remove the source of the problem, when possible.
  • To minimize and manage potential complications of ILD.
  • To improve or prevent deterioration in a patients quality of life

Because current therapy is not thought to reverse scarring that has already taken place, it is important to diagnose and treat ILD as early as possible. If your doctor has identified an occupational or environmental exposure, removal from the source of the problem is essential—even if it means giving up your job, hobby, or changing where you live. People who respond well to therapy generally report less shortness of breath with exercise and stable breathing tests.

What are some of the medications used to treat ILD?

Corticosteroids

Prednisone, or some other form of corticosteroid, is frequently the first medication used. For some people, corticosteroids alone may decrease lung inflammation and cause an improvement in symptoms. Corticosteroids can have significant side effects.

Some of the side effects include:

  • increased appetite
  • weight gain
  • high blood pressure
  • salt and fluid retention
  • tendency to bruise easily
  • depression
  • psychosis or hyperexcitability
  • tendency to develop diabetes
  • peptic ulcers
  • infections
  • cataracts
  • osteoporosis (a tendency to break bones)

Talk with your healthcare provider about preventing and watching for these side effects.

Cyclophosphamide (Cytoxan®)

Cyclophosphamide may be used in conjunction with corticosteroids to treat ILD. This medication reduces inflammation by killing some inflammatory cells and suppressing their function. This medication may require up to six months or longer to show improvement.

Some side effects of cyclophosphamide include:

  • gastrointestinal irritation
  • bladder inflammation
  • bone marrow suppression
  • infection
  • irregular menstruation
  • blood disorders

Azathioprine (Imuran®)

Azathioprine is another drug often used in combination with corticosteroids for the treatment of ILD. Azathioprine's side effects may include fever, skin rash, gastrointestinal irritation and blood disorders.

Because of the potential side effects of the above medications, your doctor will carefully monitor you while on therapy. The decision to treat patients with ILD involves a careful weighing of the potential risks and benefits of therapy.

What are some other therapies?

There are many investigational therapies currently being studied for the treatment of ILD. None of them has yet been proven to be beneficial. Some patients with ILD may qualify for involvement in clinical trials of these potential therapies; you should discuss this with your doctor.

Oxygen Therapy

Oxygen therapy is required for some people with ILD because of a low level of oxygen in the blood. Some may need oxygen therapy all of the time while others may need it only during sleep and exercise. Improving the level of oxygen in the blood through the use of supplemental oxygen can help relieve strain on the heart and lungs and improve symptoms of shortness of breath and fatigue.

Pulmonary Rehab

A pulmonary rehabilitation program is often recommended to help patients with ILD achieve their highest possible level of functioning. This program includes education, exercise conditioning, breathing techniques, energy saving techniques, respiratory therapy evaluation, nutritional counseling and psychosocial support.

Lung Transplant

If the above therapies fail to adequately treat ILD, lung transplant is an option for some advanced cases. With improved surgical techniques and post-transplant care, transplantation may offer improved quality of life and prolonged survival to selected patients.

This information has been approved by Kevin Brown, MD (January, 2005).

Note: This information is provided to you as an educational service of National Jewish. It is not meant to be a substitute for consulting with your own physician.

© Copyright 2008 National Jewish Medical and Research Center

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