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 about oxygen therapy?
What about pulmonary rehabilitation?
What about lung transplant surgery?
How should I expect to respond to treatment?

 

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 In bronchoalveolar lavage, a small amount of sterile saline is placed in one area of your lung and then withdrawn.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?

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.

The goals of treatment are to:

• Identify the specific disease, start therapy early to decrease inflammation and prevent further lung scarring.
• Remove the source of the problem, when possible.
• Minimize and manage potential complications of ILD.
• Improve or prevent deterioration in a patients quality of life.

 

What types of therapy are available?

The type of medication and length of therapy depends on the specific type of interstitial lung disease. With some types of ILD, removing the source of exposure - including cigarette smoke, some medications, and environmental irritants - may be all that is needed. With other types of ILD, medications and even surgery may be required.

Common treatment options include:

• Medications 
• Oxygen Therapy
• Pulmonary Rehabilitation
• Lung Transplant (in some advanced cases)  

 

Medications

Corticosteroids (Antifibrotic / Anti-Inflammatory Drugs)
Cytotoxic Agents or Immunosuppressive Drugs
Azathioprine (Imuran®)
Mycophenolate (CellCept®)

Corticosteroids (Antifibrotic / Anti-Inflammatory Drugs)

Oral 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. Other people may have to use steroids in combination with other therapies. It may take as long as three months to see results. 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.

Cytotoxic Agents or Immunosuppressive Drugs

Cyclophosphamide (Cytoxan®) may be used if steroid therapy has failed to be effective or if corticosteroid treatment is not possible. In some cases, a combination of a corticosteroid and cyclophosphamide is used with good results. This medication reduces inflammation by killing some inflammatory cells and suppressing their function. Response to therapy may be slow and require up to 6 months or longer. Cyclophosphamide can have significant side effects.

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. It is used if the side effects of other drugs are not tolerable. Though early studies show this drug may not be as effective, its side effects may be more manageable.

Some side effects of azathioprine may include:

• fever
• skin rash
• gastrointestinal irritation
• blood disorders.

Mycophenolate (CellCept®)

Mycophenolate can be used to help reduce the amount of steroids required. It works to prevent the immune system from attacking cells in the body that result in fibrosis. Myophenolate may produce side effects.

The most common side effects include:

• abdominal distress
• sleepiness
• muscle or joint pain 

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. The potential benefits from the treatment usually outweigh the risk from the medications side effects.

 

Oxygen Therapy

Oxygen therapy is required for some patients with ILD. Decreased lung function and/or pulmonary hypertension may cause blood oxygen levels to be too low. Some may need oxygen therapy all of the time while others may need it only during sleep and exercise. There are many systems used to deliver oxygen. They allow patients to be active and travel while using oxygen. The high altitude in Denver may result in you needing to oxygen while here. You will be tested for this and arrangements can be made for oxygen usage while you are here. 

 

Pulmonary Rehabilitation

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.

Learn more about pulmonary rehabilitation at National Jewish Health.

 

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. In Denver, lung transplants are performed at the University of Colorado Hospital (Department of Surgery) .

 

How should I expect to respond to treatment?

Response to therapy varies widely. Some types of interstitial lung disease may respond quickly and others may not respond at all. Treatment is considered successful if symptoms, physiological, and x-ray findings are stabilized. Even with treatment, many types of interstitial lung disease progress naturally with a worsening of symptoms, x-ray, and physiologic findings. Sometimes worsening is due to a complication of the disease or therapy. This may include conditions such as pulmonary hypertension or right heart failure. Some therapies may result in infection, muscle weakness and osteoporosis.

 

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