Restrictive Lung Disease involves diseases of the chest wall, pleura, and respiratory muscles. They lead to lung restriction and breathing failure. Other health problems can also restrict breathing, including a twisted spine (kyphoscoliosis), pulmonary embolisms, and morbid obesity.
A doctor who suspects a person has restrictive lung disease might order a pulmonary function test. This noninvasive technique measures lung volume, capacity, and rates of exchange.
Symptoms
Restrictive lung disease prevents the lungs from expanding fully, resulting in shortness of breath, coughing and chest pain. Many of these diseases are incurable, but there are treatment options that can help with symptoms and improve quality of life.
Physicians divide restrictive lung diseases into two categories, intrinsic and extrinsic. Intrinsic lung diseases occur within the pulmonary parenchyma, which includes alveoli (small air tubes), capillaries and interstitium. Extrinsic lung diseases occur in the pleurae, muscles of the chest wall and ribs, neuromuscular disorders and extra-pulmonary conditions that restrict pulmonary function.
Inflammation and scarring cause the lungs to become stiffer and less elastic, decreasing how much air they can hold. This can be caused by a variety of diseases, including cancer chemotherapy drugs and radiation exposure. It can also be caused by exposure to inorganic dust, such as silicosis, asbestosis and berylliosis. Diseases such as sarcoidosis and fibrosis of the lungs can also cause restrictive lung disease.
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Most people with fibrotic lung disease have symptoms such as wheezing, shortness of breath and coughing. The severity of the symptoms depends on the type and extent of the disease. Some people may need oxygen therapy to help them breathe more easily. This can be a lifesaving treatment, but it comes with its own side effects. For example, people with oxygen therapy are at risk for infections, such as pneumonia. They can also be more prone to bleeding in the lungs and heart problems.
Other treatments for fibrotic lung disease include corticosteroid medications, such as prednisone. These can be taken orally or intravenously. However, they can have a wide range of side effects, including thinning of the bones and weight gain. They can also affect blood pressure, which can increase the risk of a blood clot in the legs or lungs.
Some patients who are severely ill with restrictive lung disease may need to have a lung transplant. Usually, the new lungs come from an organ donor who has recently died. People who receive a lung transplant are required to take immunosuppressant drugs to keep the body from rejecting the new organ.
Diagnosis
During each respiratory cycle, the lungs inflate and deflate through a series of muscular contractions of the respiratory, diaphragmatic, and external intercostal muscles. The lungs inflate to the functional reserve capacity (FRC), which is the volume of air that can be expanded during a forced expiration after full inspiration. Restrictive diseases decrease FRC and total lung capacity as a result of pathology in the pulmonary parenchyma, pleura, or chest wall and neuromuscular system. The reduction is accompanied by reduced gas transfer and symptoms such as shortness of breath, cough, and wheezing.
The diagnosis of restrictive lung disease is similar to that of obstructive disorders, and usually begins with a physician’s examination. Patients with a suspected restrictive lung disease are evaluated by performing pulmonary function tests, such as spirometry, which measures the amount of air that is able to be blown out in a forced expiration. The test can help determine not only whether the person is experiencing restrictive lung disease but also the extent of the condition. Other diagnostic procedures include a physical exam, blood testing, and chest radiography. A bronchoscopy may be recommended, which involves inserting an endoscope into the lungs, allowing physicians to visualize the interior of the lungs and take tissue samples.
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A patient with a restrictive disorder may develop a buildup of fluid in the lungs, a condition called pleural effusion. This can make breathing more difficult, and is caused by infections such as pneumonia or viral illnesses such as influenza. Depending on the cause of the pleural effusion, treatment may involve antibiotics or steroid medications. A pleural effusion may also be the result of conditions such as sarcoidosis or lymphangioleiomyomatosis.
A doctor may recommend a lung transplant when the symptoms of restrictive disease are severe and not responding to other treatments. In a lung transplant, the patient receives one or both of the donor’s lungs. Those who undergo a lung transplant must also be on immunosuppressant medication to prevent the body from rejecting the organ. This major surgery can improve quality of life in some people with restrictive lung disease, but it is not a cure.
Treatment
In people with restrictive lung disease, the lungs cannot expand as well as they should. This prevents oxygen from reaching the lungs and other organs through the bloodstream. It also can cause a ventilation-perfusion mismatch that leads to hypoxemia (low levels of oxygen in the blood) and hypoventilation (difficulty breathing).
The condition may be caused by anything that causes stiffness in the lungs themselves or in the chest wall or chest and respiratory muscles. It can also occur because of nerve damage that restricts how much the lungs expand. People who smoke are at a higher risk for developing this condition. Other possible causes include exposure to certain dust particles in the workplace (e.g., silicosis, asbestosis, coal worker’s pneumoconiosis, beryllium disease, and hard metal fibrosis), and certain illnesses and treatments including chemotherapy, radiation, and rheumatoid arthritis.
Treatment options include drugs to relieve inflammation and other medications that slow the progression of the illness. Pirfenidone and nintedanib, which are both FDA-approved to treat idiopathic pulmonary fibrosis, work on multiple pathways to prevent the lung tissue from scarring. They can improve pulmonary function tests and decrease the symptoms of the disease.
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Other medications to reduce swelling and inflammation in the lungs include corticosteroids, azathioprine, and mycophenolate mofetil. Drugs that suppress the immune system, such as cyclophosphamide and methotrexate, may be used for inflammatory conditions including sarcoidosis and lupus.
Some people with restrictive lung disease need supplemental oxygen to help them breathe. Oxygen therapy is usually given through a tube that’s guided into the lungs and attached to a machine that helps you breathe. If your condition gets worse, a healthcare professional might recommend that you get mechanical ventilation in the hospital.
Pulmonary rehabilitation programs that incorporate exercise training and education on breathing techniques can improve results on pulmonary function tests, increase exercise endurance, and lower the need for oxygen. In addition, it may reduce the symptoms of the underlying disease and improve quality of life. It is important to avoid smoking and any other activities that can exacerbate your symptoms. A pulmonary doctor can recommend the best lifestyle changes to accommodate your disease and symptoms.
Prevention
People with restrictive lung disease have a difficult time getting enough oxygen because their lungs can’t expand as easily. It’s important to see your doctor if you have this condition. They will diagnose you with pulmonary function tests. During these tests, you blow air forcefully through a mouthpiece while a machine records the volume and flow of the air. The test can tell you what kind of restrictive lung disease you have and how severe it is. Your doctor may also do a physical examination and ask about your smoking history. They will also order X-rays and CT scans of your lungs.
Restrictive lung disease is caused by diseases that affect the internal structure and functioning of your lungs, called intrinsic lung disease. These conditions are primarily due to scarring and narrowing of the airways inside your lungs, which causes them to work less effectively.
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There are also a number of other things that can cause restrictive lung disease. These include obesity, obesity hypoventilation syndrome, interstitial pulmonary disease (idiopathic pulmonary fibrosis, lung cancer), eosinophilic pneumonia, and sarcoidosis. It is also possible to develop this disease from certain exposures, such as talc, hard metal, and organic dusts (farmer’s lung, mushroom worker’s lung, bird fancier’s lung) and from medications (eg, cyclophosphamide, bleomycin, hydralazine, amiodarone).
If you have an intrinsic lung disease, your doctor will treat the underlying condition that’s causing it. This might include taking corticosteroids, which help keep your lungs healthy by decreasing inflammation. However, these drugs can have serious side effects, including high blood pressure and increased risk of infection. Researchers are working to find better drugs that have fewer side effects.
In severe cases, your doctor may recommend oxygen therapy. This therapy helps you get enough oxygen when your lungs can’t expand. You might need to use oxygen only when you’re exerting yourself or when you’re sleeping. If other treatments don’t work, you might need to have a lung transplant. In a lung transplant, you receive new lungs from an organ donor who has recently died. Your doctors will probably prescribe immunosuppressants to prevent your body from rejecting the transplanted lungs.
