Treatment for ILD is based on the specific ILD diagnosis and the severity of disease.
Sometimes, an approach of watchful waiting without drug therapy is appropriate. Many forms of ILD can be treated successfully with medications. Some medications are only appropriate for certain forms of ILD and not others.
Regardless of the cause of ILD, the goals of treatment are:
To decrease inflammation and prevent the formation and/or progression of 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 person's quality of life.
Medications for ILD
There are a handful of medications that might be prescribed to people with ILD.
Prednisone or some other form of corticosteroid is used in many forms of ILD. 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 hyper-excitability and difficulty sleeping. There also is a tendency to develop diabetes, peptic ulcer, infections, cataracts and osteoporosis (weakening of the bones).
Immune suppressing or steroid sparing medications
These medications are sometimes prescribed along with or in place of corticosteroids. Each has its own side effect profile and requirements for monitoring. Some of the most commonly prescribed immunosuppressive drugs used in patients with ILD include the following:
Two medications have been approved by the U.S. Food and Drug Administration for the treatment of people with idiopathic pulmonary fibrosis (IPF). They are not approved for use in any other form of ILD.
Pirfenidone is an oral medication taken three times daily. The specific mechanism is unknown, but available data suggest it inhibits transforming growth factor beta, and it seems to have both anti-fibrotic and anti-inflammatory properties. Pirfenidone slows the progression of disease for some people with IPF.
Some side effects include:
Nausea, loss of appetite, stomach upset and photosensitivity (a tendency to easily develop sunburn; this may be severe).
Nintedanib is an oral medication taken twice daily. It is a triple kinase inhibitor that blocks several pathways that lead to the development of scars. Like pirfenidone, nintedanib slows the progression of disease for some people with IPF.
Some side effects include:
Diarrhea and, less often, nausea and vomiting.
Because of the potential side effects of the above medications, your doctor will carefully monitor you while on therapy. This will include routine bloodwork. The decision to treat people with ILD involves a careful weighing of the potential risks and benefits of therapy. The potential benefits from the treatment usually outweigh the risks from the medication side effects.
Oxygen is required for some people with ILD because of low level of oxygen in the blood. Some may need oxygen therapy all of the time while others may need it only during sleep or 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.
There often is a stigma associated with oxygen. Many people are embarrassed and are concerned with how it will look and worry about how it will change their lifestyle. There are different options for oxygen systems, and people are still able to get out their homes and even travel. Most people find once they are on oxygen, they are able to be more active, as they are not as short of breath. National Jewish Health can discuss what oxygen system will best fit your lifestyle.
A pulmonary rehabilitation program is recommended for all patients with ILD. This program includes education, exercise conditioning, breathing techniques, and energy saving techniques, respiratory therapy evaluation, nutritional counseling and psychosocial support.
The specific goals of pulmonary rehabilitation are to improve quality of life by: decreasing respiratory symptoms and complications, encouraging self-management and control over daily functioning, improving physical conditioning and exercise performance, improving emotional well-being and reducing hospitalizations.
Lung Transplant: Is it right for you?
Response to therapy varies widely. Some types of ILD may respond quickly, and others may not respond at all. Treatment is considered successful if symptoms, physiologic findings and X-ray findings are stabilized. Even with treatment, many types of ILD progress naturally with a worsening of symptoms, X-ray findings 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.
ILD may progress despite therapy. If this happens, lung transplantation may be an option for you. Lung transplant surgery replaces one or two diseased lungs with healthy lungs from a non-living donor. With improved surgical techniques and post-transplant care, this may offer you improved quality of life and longer survival. ILD doesn’t recur in transplanted lungs.
Lung transplantation is only performed at specialty medical centers. Your health care team may determine that lung transplant is the best option for you and that you are healthy enough for surgery. After an extensive evaluation, appropriate candidates are placed on a waiting list. A position on the waiting list is determined by disease severity. Wait times vary from transplant center to transplant center.
For transplant centers nearest to you, visit the Organ Procurement and Transplantation Network website.