Restrictive lung disease refers to a group of lung diseases that prevent the lungs from fully expanding with air. This restriction makes breathing difficult.
Many forms of restrictive lung disease are progressive, getting worse over time. However, some causes of restrictive lung disease can be reversed.
Restrictive lung disease treatment focuses on eliminating the causes, improving quality of life, slowing progression of the disease, and preventing complications.
The main treatment for restrictive lung disease is supportive oxygen therapy. Oxygen therapy helps people with lung diseases get enough oxygen, even when their lungs cannot fully expand.
Some people may need oxygen only at night or after exerting themselves. Others need oxygen all or most of the time.
Oxygen therapy is safe if used appropriately but can cause side effects. These side effects include:
Very rarely, too much oxygen can lead to an oxygen overdose. Oxygen is also flammable, so people receiving oxygen therapy must reduce the risk of an explosion or fire, including not lighting candles or smoking near an oxygen tank.
Some other treatment options may be available, depending on what has caused the disorder.
For instance, someone with a condition called scoliosis that restricts lung capacity must treat the scoliosis to see results.
Some treatments that might help intrinsic restrictive lung disease, which is caused by a problem with the lungs themselves, include the following:
Corticosteroids, such as prednisone, can suppress the immune system, reduce inflammation, and slow the course of both pulmonary fibrosis and sarcoidosis. However, steroids can also cause numerous side effects. These include:
- changes in mood and behavior
- weight gain
- shortness of breath
- changes in thinking
The dose and duration of corticosteroid therapy will depend on the severity of the lung disease and whether these side effects occur.
Doctors are continually researching alternative medications that can treat restrictive lung disease without as many side effects.
A drug that suppresses the immune system, called azathioprine, has helped some people, but research has not yet proven that it works.
Mycophenolate mofetil helps regulate the immune system and may improve symptoms of pulmonary fibrosis, sarcoidosis, and some other autoimmune conditions.
Methotrexate, which also suppresses the immune system, can treat inflammatory forms of restrictive lung disease, for example, sarcoidosis.
Other medications, including anti-inflammatory drugs, proton pump inhibitors, and anti-fibroid drugs, may also help.
The right drug to use depends on the cause of the restrictive lung disease and the symptoms, as well as a person's overall health, as many medications can lead to serious side effects.
Chemotherapy may help reduce inflammation caused by a restrictive lung disease called pulmonary fibrosis. However, chemotherapy also kills healthy cells and can prompt a range of side effects, such as nausea, hair loss, and a weakened immune system.
Some studies have also found a link between chemotherapy and lung damage. As such, it is important to weigh the potential benefits of chemotherapy against its risks.
A ventilator is a device that helps the lungs take in oxygen. Unlike oxygen therapy that is given through the nose, a ventilator uses a tube in the throat or a high-pressure mask to support breathing. People who have an advanced restrictive lung disease may need a ventilator to be able to breathe.
For some people, ventilator therapy is unsafe or cannot be used. Hence, in extreme cases, an alternative called extracorporeal membrane oxygenation (ECMO) delivers oxygen directly to the blood.
For ECMO, a doctor removes blood through a large vein. The blood is pumped through a membrane that provides it with oxygen, and it is then put back in the body.
ECMO requires a hospital stay, can cause serious complications, and is only appropriate for people with a severe disease who cannot use more conventional treatments.
During a lung transplant operation, a doctor removes a diseased lung and replaces it with a healthy one.
For people who have not improved with other treatments, a lung transplant may be a consideration, although they must be healthy enough to undergo surgery.
Lung transplants offer a chance of a healthier, longer life, but they are also highly risky. After a lung transplant, a person can develop life-threatening complications, such as organ rejection.
After a lung transplant, a recipient must take drugs that suppress the immune system. However, these drugs make transplant recipients more vulnerable to many infections. As a result, most transplant recipients must be closely monitored in hospital for several weeks.
Stem cell therapy
During stem cell therapy, a doctor injects the lungs with stem cells, which are cells that can grow into lung cells.
Research has not yet proven that stem cell therapy works, or that it is safe. However, people interested in stem cell therapy may be able to participate in clinical trials that offer treatment and monitoring.
Treatment for restrictive lung disease is continually evolving. Some people with symptoms of the disease may be eligible for a clinical trial. There is no guarantee that new drugs will work, but many clinical trials have helped people get better.
Lifestyle remedies may be helpful. Pulmonary rehabilitation programs help people better understand their oxygen needs and breathe more efficiently. This input can enable them to cope better with their disease.
Some people find that dietary changes, such as eating smaller meals, help with some restrictive lung diseases, including pulmonary fibrosis.
Also, people with lung restrictions due to obesity can benefit from weight loss.
Restrictive lung diseases fall into two broad categories: intrinsic lung restrictions and extrinsic lung restrictions.
Intrinsic restrictive lung disease
Problems with the lungs themselves cause intrinsic restrictive lung disease. The underlying diseases associated with intrinsic restrictive lung disease include:
- Interstitial lung disease: This is a group of more than 100 lung tissue diseases.
- Pulmonary fibrosis: This scarring on the lungs makes it more difficult for them to expand.
- Sarcoidosis: This disease causes inflammatory cells that fight infections to develop and harm the body.
- Pneumoconiosis: Pneumoconiosis occurs when tiny particles, such as dust, damage the lungs.
Extrinsic restrictive lung disease
Problems outside of the lungs cause extrinsic restrictive lung disease. These problems place pressure on the lungs, making it more difficult for them to expand.
Some causes of extrinsic restrictive lung disease include:
- a buildup of fluid around the lungs
- some autoimmune diseases, such as myasthenia gravis
- some neuromuscular diseases, such as muscular dystrophy
Restrictive vs. obstructive lung disease
Most chronic lung diseases are either restrictive or obstructive.
Restrictive lung diseases make it difficult for the lungs to expand completely, so making it harder for someone to inhale fully.
Obstructive lung diseases interfere with the ability of the lungs to exhale air fully. Examples include chronic obstructive pulmonary disease (COPD), asthma, and bronchiectasis.
Symptoms of the two groups of diseases are similar, and include:
- chronic cough
- shortness of breath
- weight loss
- coughing up blood or white sputum
- chest pain
A few significant differences between obstructive and restrictive lung disorders can help a doctor make a diagnosis. The amount of air people can inhale and how well the lungs can stretch are lower in those with restrictive rather than obstructive lung disease. This is measured with pulmonary function tests.
However, it is possible to have both restrictive and obstructive lung diseases at the same time. People with both conditions have significantly more trouble breathing than those with only one.
Doctors stage restrictive lung disease as mild, moderate, severe, early, and advanced. The more the disease has progressed, the worse the outlook is.
In some cases, treating an underlying cause of lung restriction, such as obesity or scoliosis, can slow or reverse the progression of the disease. When restrictive lung disease is caused by a lung condition, however, it is usually difficult to treat and eventually fatal.
Life expectancy depends on several factors, the most significant being how severe the disease is. One way that doctors assess outlook and life expectancy is with the GAP Index. This tool looks at age, recent respiratory hospitalizations, and how much air the person can take in.
An online calculator can help provide estimates for outlook and life expectancy. However, people should remember that estimates are exactly that, and that the best source of information on their disease is a doctor, particularly a lung specialist.