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Treatments and drugs

Interstitial lung disease caused by toxins or drugs can sometimes be reversed when you're no longer exposed to those substances. But in people for whom this isn't the case, the outlook is less promising. That's because the drug therapies that are currently available can have serious side effects and often aren't effective.

Treatment for interstitial lung disease often includes a combination of the following medications:

• Corticosteroid drugs. These anti-inflammatory drugs help a minority of people with interstitial lung disease. Those most likely to benefit have a disorder with a known cause and reversible changes in their lungs. Corticosteroids seldom improve lung function in people with idiopathic pulmonary fibrosis, and when they do, the benefits are usually temporary.

  Taken for long periods of time or in large doses, corticosteroids can cause a number of side effects, including glaucoma, bone loss, high blood sugar levels leading to diabetes, poor wound healing and increased susceptibility to infection.

• Azathioprine. This cytotoxic medication, which is normally used to prevent organ rejection after a transplant, may also be used in combination with corticosteroids to treat interstitial lung disease. Azathioprine can cause severe side effects, including reduced production of red blood cells and an increased risk of infection and certain cancers. Another cytotoxic drug, cyclophosphamide, may be tried if azathioprine is not effective. However, the side effects associated with cyclophosphamide are even more severe.
• Acetylcysteine. This medication belongs to a class of drugs called antioxidants. Oxidation is a natural process that leads to cell and tissue damage. The process may play a role in the development of scarring in the lungs (pulmonary fibrosis). Results from a clinical trial found that, when combined with corticosteroids and azathioprine, acetylcysteine improved lung function in people with idiopathic pulmonary fibrosis. No significant changes were seen in mortality rates.
• Anti-fibrotics. These drugs are sometimes used to help reduce the development of scar tissue. In clinical studies, the medications bosentan and pirfenidone have shown promise for slowing the progression of lung damage. Follow-up studies to gain more information about the risks and benefits of these drugs in the treatment of interstitial lung disease are under way. Other anti-fibrotic agents that have been studied as possible treatments for this condition — including penicillamine, colchicine and interferon gamma-1b — have not been found effective.
• Oxygen therapy. Depending on the severity of your symptoms and your activity level, your doctor may recommend oxygen therapy. Although oxygen can't stop lung damage, it can make breathing and exercise easier and prevent or lessen complications from low blood oxygen levels. Oxygen therapy may also improve your sleep and sense of well-being. It can also reduce blood pressure in the right side of your heart.

• Pulmonary rehabilitation. This is a formal program for people with chronic lung disease that includes, but goes far beyond, medical management. The aim of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning, but also to help people with pulmonary fibrosis live full, satisfying lives. Pulmonary rehabilitation programs focus on exercise, teaching you how to breathe more efficiently, education, and emotional support and nutritional counseling.

  Most often, this multifaceted approach requires a team of health care providers that may include a doctor, nurse, rehabilitation specialist, dietitian and social worker. Programs can vary widely, however. Your doctor can usually tell you about pulmonary rehabilitation programs in your area. Or contact the American Lung Association at 800-LUNGUSA (800-586-4872) for more information.

• Lung transplantation. This may be an option for people with severe interstitial lung disease who aren't likely to benefit from other treatment options.

  In order to be considered for a transplant, you must agree to quit smoking if you smoke, be healthy enough to undergo surgery and post-transplant treatments, be willing and able to follow the medical program outlined by the rehabilitation and transplant team, and have the patience and emotional strength and support to undergo the wait for a donor organ. The last is particularly important because donor organs are in short supply.

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