Interstitial lung disease

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. Treatments for interstitial lung disease include:

• Corticosteroid drugs. Although these anti-inflammatory drugs are the initial treatment of choice, they help a minority of people with interstitial lung disease. Those most likely to benefit have a nonidiopathic disorder 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.

  In general, you take corticosteroids for several months until symptoms improve and then you slowly taper off the medication. If your symptoms return, your doctor may recommend further corticosteroid therapy or an immunosuppressive drug such as azathioprine. 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.

• Cytotoxic drugs. Azathioprine, which is normally used to prevent organ rejection after a transplant, and the anti-cancer drug cyclophosphamide may be used to treat interstitial lung disease. The drugs are prescribed when corticosteroids fail to improve symptoms or, increasingly, as a first-line treatment in combination with steroids. Cytotoxic drugs can cause severe side effects, including reduced production of red blood cells and an increased risk of infection and certain cancers.
• Anti-fibrotics. These drugs — such as bosentan, colchicine, interferon gamma-1b, penicillamine and pirfenidone — are sometimes used to help reduce the development of scar tissue. In clinical studies, they showed promise for slowing the progression of lung damage without suppressing the immune system, but real-world results have generally been disappointing.
• 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). One trial of the antioxidant acetylcysteine found modest improvements in lung function in people with idiopathic pulmonary fibrosis, though no significant changes were seen in mortality rates.

• 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.

  You're most likely to receive oxygen therapy when you sleep or exercise, although some people may use oxygen therapy around the clock. Children with interstitial lung disease also are likely to need oxygen therapy.

• 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. To that end, pulmonary rehabilitation programs focus on exercise, on teaching you how to breathe more efficiently, on education, and on 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, or 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.

  In general, single-lung transplants are more successful in people with interstitial lung disease than double-lung transplants are.