Interstitial Lung Disease
Interstitial Lung Disease Overview
Interstitial lung disease refers to a broad selection of lung diseases in which the interstitium—the tissue surrounding and separating the tiny air sacs (alveoli) in the lungs—becomes inflamed, stiff or scarred. When this occurs, the air sacs cannot efficiently expand with each breath, thus restricting the amount of oxygen circulating through your lungs and your body.
Physicians believe that some interstitial lung diseases occur when an infection, autoimmune disease, injury, medications, toxins, radiation therapy or some other stimulus triggers an abnormal healing response. Normally, your body generates the proper amount of tissue to repair the damage, but with this disorder, your body produces excess scar tissue, which may impair normal lung function.
Often, your Wellstar physician will be able to determine the initial cause of interstitial lung disease. However, in many cases the cause is not known. Disorders without an identified cause are referred to as idiopathic (unknown) interstitial lung disease. Similar to interstitial lung diseases of known origins, the idiopathic varieties are categorized according to location, severity and pattern of lung involvement. Idiopathic pulmonary fibrosis is the most common of these disorders.
Symptoms
The chronic symptoms of interstitial lung disease may include:
- Shortness of breath
- Dry cough
- Wheezing
- Chest pain
As the symptoms worsen, you may even notice you’re becoming breathless during the most routine activities such as getting dressed or eating. You may experience weight loss and fatigue. In more advanced stages of interstitial lung disease, you may experience cyanosis, a blue coloring of the lips, skin or fingernails due to the lack of oxygen; clubbing, abnormal enlargement of the fingernails; or pulmonary hypertension, a type of high blood pressure that affects the arteries within the lungs.
Risk factors
Interstitial lung disease typically affects adults, although infants and children may develop the disorder. Several other factors make you more susceptible to interstitial lung disease. These include:
- Exposure to toxins: If your work or living arrangements expose you to pollutants that are known to damage your lungs (asbestos, silica dust, bacteria or fungal overgrowth, agricultural grains and dust), your risk increases greatly.
- Radiation and chemotherapy: Radiation treatments to your chest and some chemotherapy drugs increase your chances for some form of this disorder.
Specific to idiopathic interstitial lung disease, researchers believe several factors put you at risk, including smoking, genetic factors and gastro esophageal reflux disease, where stomach acids back up into your esophagus and get drawn into your lungs.
Interstitial Lung Disease Prevention
In general, avoid or limit exposure to toxins or treatments that can lead to interstitial lung disease.
Reduce your risk for interstitial lung disease
Quitting smoking and avoiding exposure to substances known to cause lung disease can prevent the disorder from developing or worsening. People who are employed in jobs where they may be heavily exposed to known causes of lung disease in the workplace typically undergo routine screening for lung disease.
Tests and screenings
Interstitial lung disease can be difficult to diagnose because an unusually large number of disorders fall into this category. In addition, the distinction between known causes and unknown causes of the disease is not always clear. Because the symptoms of a number of other pulmonary and heart diseases—such as chronic obstructive pulmonary disease (COPD), heart failure and asthma—mimic those of interstitial lung disease, your Wellstar physician needs to rule those out before making a diagnosis.
To diagnose interstitial lung disease, your Wellstar physician will take a careful medical history and perform a physical examination. When interstitial lung disease is suspected, your physician may order a variety of tests or procedures:
- Imaging, such as a chest X-ray or computerized tomography (CT) scan to eliminate other possible conditions causing signs and symptoms like those of interstitial lung disease and to reveal details of your lung tissue
- Blood tests to check for connective tissue diseases
- Lung function tests that measure the volume of your lungs, the amount of air you breathe in and out, the rate of your breathing and the ability of your lungs to deliver oxygen to your blood
- Exercise tests to test lung function
- Bronchoscopy or trans bronchial biopsy, a procedure in which your physician passes a fiber-optic tube called a bronchoscope through your mouth or nose and into your lungs to remove tissue samples for examination
- Bronchoalveolar lavage, a procedure in which your physician inserts a bronchoscope into your lungs, injects a saline solution and immediately suctions it out—the collected liquid is then sent to the lab for examination
Interstitial Lung Disease Diagnosis
If screening tests or check-ups reveal abnormal results, your Wellstar physician will perform further evaluations regarding interstitial lung disease.
Procedures
The tests may include some of the following:
- Chest X-rays
- Computerized Tomography (CT) Scan
- Blood tests
- Lung function tests (pulmonary function tests/PFTs)
- Exercise tests
- Bronchoscopy with biopsy
- Bronchoalveolar lavage
- Video-assisted thoracoscopic surgery
Interstitial Lung Disease Treatment
In some cases of interstitial lung disease, the symptoms can be reversed simply by eliminating exposure to the toxins or drugs that initially cause the disorder. In more advanced cases, damaged lung tissue cannot be reversed and requires medication, therapy, surgery or even a lung transplant. Treatment depends on the cause, if known, and severity of the disease. The cessation of smoking, however, is essential in all forms of treatment.
Medication
Medications currently available for interstitial lung disease typically work by controlling inflammation in the lungs. Most commonly, corticosteroid drugs are given in an attempt to reduce inflammation. However, when taken for long periods, they can cause glaucoma, bone loss, high blood sugar levels and susceptibility to infection. Other drugs—azathioprine, mycophenelate and rituximab—may help reduce development of scar tissue and other infections, but come with risks of other serious side effects. More recently, a class of medication known as “anti-fibrotics” has been approved for idiopathic pulmonary fibrosis and other progressive fibrotic interstitial lung diseases. These have been shown to significantly slow progression of lung disease.
Therapy
Therapy for interstitial lung disease is designed specifically to make you more comfortable and to improve lung function. Oxygen therapy cannot stop lung damage, but it can make breathing and exercise easier and prevent or lessen complications due to low oxygen levels. Pulmonary rehabilitation is designed to treat the disease and to improve daily functioning. This program, usually administered by a team of Wellstar pulmonary professionals, provides support, education about proper breathing methods, energy-saving habits and nutrition and diet guidelines.
Surgery
In rare cases, your Wellstar physician may recommend a lung transplant if you would not benefit from any other treatment options.
Ongoing Care for Interstitial Lung Disease
Wellstar’s world-class, community-based physicians utilize state-of-the-art medical centers and hospitals with the latest technologies and medical resources. Our clinicians are dedicated to:
- Helping you create and maintain healthy lifestyles
- Offering advice to prevent illness and injuries
- Providing early and appropriate care of acute illness to prevent its progression
Pulmonary Medicine
We provide top-flight pulmonary care throughout Wellstar Health System. Each of Wellstar’s physicians, certified in their field, have the knowledge and skill needed to diagnose and treat all pulmonary conditions and diseases, ranging from asthma, bronchitis, emphysema, pneumonia, respiratory failure, pulmonary hypertension, lung cancer and sleep apnea. In addition, many of our providers have special training in internal medicine, pulmonary diseases and critical care medicine. As a result, you can feel confident—at every level—about your pulmonary care at Wellstar.
Ongoing care at home
Proper care and treatment of interstitial lung disease can often improve symptoms and help you live longer. You and your Wellstar physician can work together to make your life more comfortable, so pay close attention to your body and your post-treatment regimen and keep your doctor updated. As part of your post-treatment care and in addition to maintaining a healthy diet and exercise, you should:
- Stop smoking
- Seek prompt medical attention when symptoms increase in severity
- Keep track of all medications you take
- Keep track of your weight and blood pressure
- Write down your questions, and never be afraid to ask your Wellstar physicians for clarification regarding your condition or your treatment