Interstitial Lung Disease - Symptoms, Causes and Treatment
In most cases, coughing might not be just a cough. It can be a severe lung disease such as interstitial lung disease that would require urgent and critical attention.
Interstitial lung disease (ILD) progresses quietly with the usual symptoms such as coughing and shortness of breath, which you can easily ignore. You may realize its existence at its later stages, which might be challenging to manage the symptoms.
What may surprise you is that interstitial lung disease has a life expectancy of between 3 and 5 years from its onset.
What is Interstitial Lung Disease?
Interstitial lung disease is not a disease but a name for several lung diseases that affect the lung interstitium (lung support tissue) or interstitial space. ILD causes inflammation and then scarring of the lung tissue.
There are over 200 types of lung diseases under the interstitial lung disease umbrella.
Any lung condition that affects the interstitium tissue is called interstitial lung disease. Most of the ILD types cause scarred interstitium, which may result in lung fibrosis.
The interstitium is a light net-like tissue that allows gas exchange from the alveoli. When the interstitium is scarred either from inflammation or an injury, it affects the air sacs' functions. The air sacs (alveoli) with fibrosis can fail to exchange the two gases, oxygen and carbon dioxide, to and from the blood.
How Interstitial Lung Disease Occurs
Interstitial lung disease occurs as the lung tissue, which is the interstitium, forms some fibrosis when healing from inflammation or injury. The interstitium cells repair abnormally to create thick and scarred tissue with fibrosis.
The remodeling of the injured tissue in a normal healing process happens without the tissue piling up on one another to cause fibrosis. The cells grow and multiplicate with the right mass of tissue to repair the damaged part.
With healthy lungs, the oxygen from the alveoli enters into the capillaries through the interstitium. On the other hand, a scarred interstitium prevents the oxygen from passing through it and into the bloodstream.
Interstitial lung disease is a progressive condition that can also affect the lungs to reduce in size, change shape, and even to become stiff. When the lungs distort their shape, they affect the airways to lose their shape as well.
The scarring and the interstitium damage, plus the air sacs, and the capillaries, are permanent and irreversible. However, there are some treatments to manage the symptoms and the disease.
Types of Interstitial Lung Disease
As we had mentioned, interstitial lung disease is a name of more than 200 types of lung diseases that affect the lung parenchyma (the airways, alveolar ducts, and alveoli).
The most common disease in this interstitial lung disease group is another sub-group known as idiopathic interstitial pneumonia. The sub-group includes a highly fatal disease called idiopathic pulmonary fibrosis.
About five primary interstitial lung disease classifications and other types are not in the categories that make up the many ILD types. The categories include ILD types of exposure-related, treatment-related, idiopathic, autoimmune, and sarcoidosis.
The following are some of the interstitial lung disease types:
Asbestosis. Asbestosis is an exposure-related lung disease that results from inhaling asbestos fibers. It causes lung inflammation and scarring.
Hypersensitivity Pneumonitis. Hypersensitivity pneumonitis is exposure-related lung disease from inhaling fungal allergens, and other irritants can cause alveoli inflammation.
Bronchiolitis Obliterans. It is a lung condition that affects and blocks the bronchioles, the smallest lung's airways, and the alveoli. It is also called cryptogenic organizing pneumonia (COP), which causes inflammation to the lung parenchyma.
Idiopathic Pulmonary Fibrosis. Idiopathic pulmonary fibrosis has more patients than the other ILD types, and it is a mysterious lung disease with unknown causes. It affects the interstitium by causing scar tissue growth.
Nonspecific interstitial Pneumonitis. Nonspecific interstitial Pneumonitis is a disease of the interstitium in the lungs. Although a rare condition, it comorbid (overlaps) other autoimmune disorders such as scleroderma, lupus, or rheumatoid arthritis.
Usual Interstitial Pneumonia. It is pneumonia commonly caused by mycoplasma pneumoniae that affects the interstitium to cause scarring. Some bacteria, viruses, or fungi can cause the condition.
Acute Interstitial Pneumonitis. It is also called Hamman-Rich syndrome. It is a severe and rapidly progressive interstitial lung disease. Acute interstitial Pneumonitis is an emergency condition that can rapidly lead to respiratory failure.
Desquamative Interstitial Pneumonitis (DIP). It is an interstitial lung disease that is closely associated with tobacco smoking. Some of the symptoms of DIP are dyspnea (shortness of breath) and coughing.
Familial Pulmonary Fibrosis. It is a family interstitial lung disease that affects two or more immediate family members.
Acute Exacerbation Interstitial Lung Disease. In worst conditions, the disease can manifest into acute exacerbation interstitial lung disease.
Acute exacerbation in interstitial lung disease (AE-ILD) progresses rapidly within a month to severe respiratory symptoms of worse dyspnea. Other severe symptoms are respiratory failure, severe hypoxemia, which would require ventilation assistance.
It is worth noting that the AE-ILD mortality rate in hypersensitivity pneumonitis of at least one month is between 75 and 100 percent after admission in the ICU. The respiratory complications in AE-ILD would require admission to the ICU for ventilation assistance and other emergency treatments.
ILD can occur at any age and even in children, but it is more prevalent with older people. The disease can gradually progress and move through stages from mild to severe. For the acute type, the disease progresses rapidly through stages to advance in a short time if not treated.
Stages of Interstitial Lung Disease
Your doctor would need to identify the stage of your interstitial lung disease before treatment. By determining the progressing level would help to give an estimation of the patient's mortality risk.
Mild. The mild stage of ILD is when it is in its initial stages. An estimate of life expectancy in this stage is 5+ years with the right treatment.
Moderate. Moderate is a stage a little bit advanced from the initial step. It means that the disease has progressed, affecting and lessening the life expectancy to between 3 and 5 years while under treatment.
Severe. The disease has immensely progressed to a severe stage and has a seriously scarred interstitium and compromised lung functions. It is a near end-stage with a life expectancy of about 3+ years while receiving the right treatment.
Advanced. It is the end-stage of the disease that causes chronic cough, dyspnea, and hypoxemia to most patients. Such symptoms are an indication of a life expectancy of fewer than three years with the right treatment.
Most of such patients in the advanced stage would be in the ICU receiving oxygen therapy for breathing support.
It is sad to realize that interstitial lung disease generally has a life expectancy of fewer than five years. You or your loved one can improve the said duration by getting the appropriate treatment at its onset or early enough.
Living a high-quality lifestyle with a carefully regulated diet and exercise can also improve your health.
Interstitial Lung Disease (ILD) vs. Chronic Obstructive Pulmonary Disease (COPD)
Both are umbrella terms for other lung diseases. Chronic obstructive pulmonary disease takes in two conditions, emphysema and chronic bronchitis. They are the two lung diseases that obstruct the lungs from functioning as they should.
COPD is an obstructive lung disease. ILD is a restrictive disease of the lungs, which means that it restricts oxygen from passing into the bloodstream due to scarring and hardening of the interstitium tissue.
While the cause of COPD is primarily smoking, little is known of interstitial lung disease with some presumptuous causes such as air pollution and genetic conditions.
Both groups of lung disease are not curable and progress in stages but with different life expectancies. A COPD patient can live beyond five years, while an interstitial lung disease patent can only live within five years.
COPD causes airways constricting or tightening that affects the inhalation and exhalation of the lungs. Coughing, wheezing, and dyspnea are some of the symptoms a COPD patient may experience.
Interstitial Lung Disease Symptoms
As mentioned earlier, interstitial lung disease is a restrictive lung condition. Some of the interstitial lung disease symptoms include:
- Dyspnea (shortness of breath)
- Dry consistent cough
- Low exercise tolerance
- Weight loss
What Causes Interstitial Lung Disease?
If you get an injury, your body forms enough tissue for repair during the healing process. It is not the same as interstitial lung disease. During the healing process, your body behaves abnormally by releasing more than required tissue, which would cause tissue pilling, which thickens the tissue while forming scars.
When the tissue around the alveoli thickens and becomes scarred, it restricts oxygen from getting into the bloodstream.
Why does interstitial lung diseases happen?
Interstitial lung disease may occur due to various causing factors, although some ILD types have no known cause. Interstitial lung disease causes include:
Viruses: Some viruses can cause interstitial lung disease. According to one study by ScienceDirect, viruses such as cytomegalovirus and human herpes virus were identified in respiratory tissue samples of some interstitial lung disease patients.
Bacteria: The most common bacteria that cause interstitial lung disease is called mycoplasma pneumoniae.
Fungi: Some fungal toxins or bird droppings can likely cause hypersensitivity pneumonitis, a type of interstitial lung disease.
Environmental toxins: Some airborne toxins such as asbestos fibers, coal dust, silica dust, and talc can damage your lungs to cause interstitial lung disease.
Health conditions: Some autoimmune diseases can exacerbate lung damage leading to interstitial lung disease. Some of these autoimmune diseases are rheumatoid arthritis, Sjogren's syndrome, scleroderma, and connective tissue diseases.
Medications: Some prescription and over-the-counter drugs can damage the lungs and hence cause interstitial lung disease. A selection of such medicines is antibiotics, some chemotherapy drugs, anti-inflammatory drugs, and some heart medications.
Interstitial Lung Disease Risk Factors
Even though anyone can get interstitial lung disease, some people are more prone to the disease because of some risk factors such as:
Smoking: Smoking damages the lungs and can risk various lung diseases, including interstitial lung disease.
Autoimmune diseases: If you have rheumatoid arthritis, lupus, and other autoimmune disorders, you are at a high risk of interstitial lung disease.
Radiation therapy: Radiation therapy can cause radiation pneumonitis, which may occur within one to three months after radiation therapy. Radiation pneumonitis, and especially the chronic type, can lead to interstitial lung disease.
Genetics: Some types of interstitial lung diseases, such as familial pulmonary fibrosis, affect immediate family members due to genetic factors.
Age: Even though interstitial lung disease can occur in babies, children, and teens as child interstitial lung disease (chILD), adults are more at risk of the disease.
GERD (gastroesophageal reflux disease): If you have chronic acid reflux, you are at risk of interstitial lung disease.
The severity of the interstitial lung disease and the rapid progression may differ with the different ILD types due to the causing factors and person to person. Despite that, it is essential to get an appropriate diagnosis as early as possible to prevent disease progression and to become worse.
Interstitial Lung Disease Diagnoses
As we mentioned earlier, some interstitial lung disease types have no known causing factors to diagnose the disease.
Another challenge while diagnosing ILD is its symptoms, such as shortness of breath and coughing, which are similar to most other lung diseases. It can, therefore, lead to wrong or late diagnosis.
Your doctor may use some of the following procedures while diagnosing your ILD case:
Very few doctors would start lung disease diagnosis without having first to examine the chest physically. Your doctor listens to your breathing by using a stethoscope to note any sounds from the lungs.
It is essential to perform this test to rule out other lung diseases that might match the symptoms.
The sure way for your doctor to make the right diagnosis is by performing imaging tests to have a clear picture of your lungs. The following are some of the image testing procedures your doctor would prefer to use:
Chest X-ray: A chest X-ray might be the first option of image testing. It is a simple procedure that would take only a few minutes to get the right image. The X-ray image would reveal the condition of the lungs by showing whiteness in the affected areas.
CT-Scan: Computerized tomography scan would be another imaging test your doctor may require for more explicit images than X-ray pictures. The images captured from different angles can help your doctor make distinctive decisions.
HRCT scan: High-resolution computerized tomography scan is an advanced imaging test than the typical CT-scan or X-ray. These images reveal any changes in the lung tissue pattern and the extent of the disease's damage to the lung tissue. The fibrosis would also come out clear in the images from an HRCT scan for further guidance in the treatment methods.
Lung Function Test
Spirometry: Spirometry is a typical test that your doctor can perform in the office. For the test, you use a tube that is connected to a measuring machine. You breathe into it while the equipment keeps records of your lung's performance.
The spirometry can record how much air you can hold in your lungs, the lungs' performance by measuring the inhaled and exhaled air, and the rate of exhalation. It also tests the functional ability of the alveoli to move the oxygen into the bloodstream.
Oximetry: Oximetry is another procedure to check on the oxygen saturation level in the blood. It is a painless, non-invasive test where your doctor places a small device at the tip of your finger. The device detects the blood saturation changes in the body. Your doctor might carry out the test in both your resting position and while active.
Lung Tissue Biopsy
Bronchoscopy: Bronchoscopy is another test that can help your doctor get into the airways to remove a tissue sample for analysis. It is different from biopsy due to the procedure and the tissue sample size, which is slightly smaller than a biopsy tissue sample.
While performing the bronchoscopy, your doctor may use a bronchoscope device to get through your mouth or nose and into your airways.
Surgical biopsy: It is a classic process for collecting the lung tissue sample. Besides being a surgical and an invasive procedure, it may be accurate in obtaining the diagnosis results.
The incision may be through your ribs to get into the lungs. The procedure has two advantages. The surgeon has the opportunity to view inside the lungs on a video with images from a tiny camera to help remove the tissue sample as well.
Iridology is a fantastic tool used to analyze areas of concern in the pleura and lungs. A skilled Iridologist can recognize many potential problems by studying the body's nerve reflexes connected to, and affecting, the eyes.
Keep in mind that Iridology is not a diagnostic tool but a tool used by a skilled Iridologist in recognizing potential problems.
After diagnosing and confirming that the disease is an interstitial lung disease, what next?
Treatment of the Interstitial Lung Disease
Interstitial lung disease is a complicated condition that is yet to have a treatment that can reverse and cure it. However, it does not mean that ILD treatment might be a waste of effort, and with no results, it can help in the following three areas:
- Improve symptoms
- Support the lungs' functioning ability
- Inhibit disease progression
- Improve your life quality
ILD treatment would also depend on the type of disease and its severity. The treatment options include:
- Inflammation and fibrosis: Oral corticosteroids (prednisone) and cyclophosphamide.
- Autoimmunity: Mycophenolate, rituximab, azathioprine, tacrolimus, and leflunomide
- Slow disease progress: Pirfenidone and nintedanib
- Stomach acid: Lansoprazole, pantoprazole, and omeprazole can help reduce stomach acid.
However, most oral medications may have some significant side effects. Discuss the treatment with your doctor to know what to expect from the treatment.
Oxygen therapy is necessary for the following factors:
- Raise the oxygen level in the blood
- Reduce blood pressure
- Assist in breathing
- Improving your sleep and health
Pulmonary exercises can improve your lung's functionalities to provide a quality life. Your doctor may suggest you do the following while performing pulmonary exercises:
- Breathing exercises
- Physical exercises
Lung Surgery - Transplantation
For advanced and severe interstitial lung disease, lung transplantation can be the last option to help prolong life. However, there are some modalities before and after the surgery you may want to discuss with your doctor.
Interstitial Lung Disease Alternative Treatment and Home Remedies
During the early stages of your interstitial lung disease, your doctor may prescribe medications to help you control and manage your symptoms. However, you may need to support your treatment with the following alternative procedures:
Be involved: It is essential to become involved in everything your doctor requires you to do. Take your medicine promptly and correctly as per the prescription.
Stop smoking: If you have been diagnosed with interstitial lung disease, it will help if you quit smoking. If you find it challenging to stop smoking immediately, your doctor can guide you on how to stop smoking.
Healthy lifestyle: Living a healthy lifestyle can help you lead a healthy life. If you are to drink alcohol, it will help if you do it moderately.
Exercises: Take time to exercise as much as you can. Involve your physical trainer to plan a suitable exercise plan that would keep you fit without straining your health.
Drinking water: Drinking plenty of water is not only healthy, but it can help you in thinning phlegm or mucus, if any, for easy coughing it out.
Healthy Diet: One of the ILD symptoms is the loss of weight. Eat a healthy and well-balanced diet. Your body needs enough calories and nutrients now more than ever to counter weight loss.
Stem cell therapy: Stem cell therapy is a regenerative treatment that can help to calm your symptoms. According to Lung Health Institute, you can improve your interstitial lung disease through cellular therapy.
Stem cell therapy (SCT) is a non-invasive procedure also useful in replacing the damaged body cells.
Stem cell therapy can promote natural healing, inhibiting ILD progression, and supporting the standard treatment.
Ozone therapy: Most of the symptoms and complications of interstitial lung disease are caused by insufficient oxygen in the blood. Ozone therapy can elevate blood oxygen levels to improve the symptoms and prevent most complications from affecting life quality.
Interstitial Lung Disease Complications
The effect of dysfunctioning lungs and insufficient oxygen in the blood can cause the following complications:
Pulmonary hypertension: The restricted blood flow in the lungs due to the affected alveoli can increase blood pressure in the pulmonary arteries. This causes pulmonary hypertension, which progresses and worsens as the ILD advances.
Respiratory failure: At an advanced stage of interstitial lung disease, low oxygen in the blood and pulmonary pressure can cause respiratory failure.
Right-heart failure: The effect of blood pressure in the pulmonary arteries leads to the heart's right side pumping harder to move blood into the lungs. The heart must work harder than usual due to the obstructing arteries in the lungs. The pressure in the right ventricle would then lead to right-heart failure.
Ischaemic heart disease (IHD): Ischaemic heart disease is common in patients with interstitial lung disease. According to one journal, 68 percent of ILD patients have ischaemic heart disease.
Interstitial lung disease is a chronic disease with no cure yet. Research and more studies are ongoing to come up with a therapy that can cure it.
It is an irreversible disease, especially when the interstitium is scarred with fibrosis. However, it is never too late. Whatever the disease stage, you can get the right treatment to slow its progress and enjoy your life moments with quality life as much as possible.
Please post your comments, questions, or insights in the comments section here below. We would be excited to read your contribution. You can also visit our Island Healthworks Natural Clinic website to learn more about other lung diseases and additional information.
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This article is intended for educational purposes and the information contained within is not intended to treat, diagnose or cure any disease or health problem. Please seek appropriate medical attention for any health complaints. We cannot take responsibility for your health care decisions. Our intent is only to offer health information to help you with your search for better health.