Interstitial lung disease (ILD)

2021-07-24 03:35 PM

Interstitial lung disease (ILD) actually describes a group of disorders, most of which cause progressive scarring of lung tissue.


Interstitial lung disease (ILD) actually describes a group of disorders, most of which cause progressive scarring of lung tissue. This has affected your ability to breathe and get enough oxygen in your blood. The disorders vary greatly.

In most cases, interstitial lung disease develops gradually, but some appear suddenly. Doctors can determine why some cases of interstitial lung disease occur, but many people don't have a cause.

In all cases, it is usually irreversible once lung scarring has occurred. Medicines can sometimes slow the damage of interstitial lung disease, but many people never regain full lung function. The researchers hope the newer drug, many of which are still experimental, could eventually prove effective in treating interstitial lung disease.


Disturbing signs and symptoms of many subtypes of interstitial lung disease may include:

Feeling short of breath, especially during or after physical activity.

Dry cough.


Chest pain.

The nail has a curve on the top (club).

Shortness of breath and a dry cough are often the initial signs and symptoms. Because these problems are vague and tend to develop gradually - often long after irreversible damage to the lungs - it can be attributed to aging, asthma, being overweight, or smoking. foliar, or long-acting forms of upper respiratory infections.

Symptoms tend to gradually get worse. You may eventually notice shortness of breath during routine activities - dressing, talking, even eating. At this point, shortness of breath becomes impossible to ignore.

By the time signs and symptoms such as shortness of breath and cough appear, irreversible lung damage has often already occurred. However, it is important to see a doctor at the first sign of shortness of breath. Many other problems with interstitial lung disease can affect the lungs, and an early and accurate diagnosis is crucial for proper treatment.


With each inhalation, air enters the lungs through large airways called bronchi. Inside the lungs, bronchi divide into smaller airways (bronchioles) that eventually end in clusters of small air sacs (alveoli). The walls of the alveoli are small blood vessels (capillaries) where oxygen is added to the blood and carbon dioxide - a waste product of metabolism - is removed.

Scars of alveolar sacs

In interstitial lung disease, the walls of the alveoli can become inflamed, and the tissue of the airways and supporting the sacs become increasingly thickened and scar tissue forms. Normally, the alveolar sacs are highly elastic, expanding and contracting like tiny bubbles with each breath. But scarring (fibrosis) causes tissue stiffness, the interstitium is thinner and thicker, making alveolar sacs less flexible. Instead of being soft and elastic, air sacs are textured like a stiff sponge, making breathing difficult and making it harder for oxygen to get into the blood through its thick walls.

Abnormal reaction

Scarring in interstitial lung disease seems to occur as spontaneous healing of an injury to the lung. Normally, the body makes some tissue to repair the damage. But in interstitial lung disease, the repair process goes wrong, creating scar tissue that increasingly interferes with lung function.

A disorder, many causes

Because interstitial lung disease has a wide range of causes, determining the reason for the initial injury to lung tissue can be difficult. Some may have multiple contributing factors including:

Labor and environmental factors. Long-term exposure to certain toxins or pollutants can lead to serious lung damage. Workers who regularly inhale silica dust, asbestos fibers, and hard metal dust are particularly at risk of serious lung disease. So people are exposed to certain chemical fumes and ammonia or chlorine gas.
Long-term exposure to a wide range of substances, many of which are organic, can also damage the lungs. Among these are grains, sugar cane, and dust from bird and animal droppings. Other substances, such as molds, can be problematic as they cause a hypersensitivity reaction in the lungs (hypersensitivity pneumonitis). Even bacterial or fungal overgrowth in hot, humid environments can cause lung damage.

Infection. These include viral infections such as cytomegalovirus, a specific virus if the immune system is weakened, certain bacterial infections including pneumonia, infectious diseases such as fungi, histoplasmosis, and parasitic infections.

Radiation. Some people who receive radiation therapy for lung cancer or breast cancer show signs of lung damage after radiation treatment ends. The severity of damage can depend on how much radiation the lungs are exposed to, the total amount of radiation received, the chemotherapy still being used, and whether the lung disease is present.

Medicine. Some medications can damage lung tissue. Those that are more likely to cause lung problems to include chemotherapy drugs, medicines to treat arrhythmias and other heart problems, certain psychiatric medications, and some antibiotics.

Other medical conditions. Interstitial lung disease can occur with other disorders. Usually, these problems don't directly attack the lungs, but instead, affect cellular metabolism throughout the body. Among these are lupus, scleroderma, rheumatoid arthritis, dermatomyositis, polymyositis, Sjogren's syndrome, and sarcoidosis. Some researchers think that gastroesophageal reflux disease (GERD) — in which stomach contents enter the lungs chronically — may lead to pulmonary fibrosis.

Unknown causes 

Although doctors can determine why some people have interstitial lung disease, in many cases the cause is unknown. Disorders without a cause are grouped together, with idiopathic pulmonary fibrosis called idiopathic interstitial lung disease. Although the idiopathic disease has some common features, each one also has unique features.

Common interstitial pneumonia, also known as idiopathic pulmonary fibrosis, is the most common of idiopathic interstitial lung disease. Because interstitial pneumonia often develops in defective lung tissue, some areas of the lung are normal, places are inflamed and still others are marked by scar tissue. This disease affects men more than women and usually develops in people over the age of 50.

Other, less common types of idiopathic pulmonary fibrosis include:

Nonspecific interstitial pneumonia.

Bronchiolitis obliterans with organizing pneumonia (Boop).

Bronchiolitis - interstitial lung.

Desquamative interstitial pneumonia.

White interstitial pneumonia.

Acute interstitial pneumonia.

Risk factors

Factors that can make you more susceptible to interstitial lung disease include:

Age. Interstitial lung disease is more likely to affect adults, infants, and children although sometimes develops these disorders. The idiopathic form of the disease usually develops in people older than 50.

Exposure to toxins at work and in the environment. If you work in agriculture, mining, construction, or for some other reason, you are exposed to pollutants known to cause lung damage, your risk of interstitial lung disease increases significantly.

Radiation and chemotherapy. Radiation treatment to the chest area or the use of certain chemotherapy drugs increases the likelihood of developing lung disease.

Oxygen. Inhaling oxygen at very high levels for more than 48 hours can damage the lungs.

Risk factors for idiopathic interstitial lung disease

Researchers have identified factors that appear to increase the risk of idiopathic pulmonary disease, although the cause of the disorder is unknown:

Smoke. Interstitial lung disease is more likely to occur in people with a history of smoking, and smoking can make the condition worse. This risk seems to increase with the number of years and number of cigarettes smoked. However, with rare exceptions, smoking does not directly cause interstitial lung disease.

Genetic factors. Rarely, idiopathic interstitial lung disease runs in families. Called familial pulmonary fibrosis, it resembles other forms of the disease. Although research is being done on familial pulmonary fibrosis, researchers have yet to identify the genes that may be responsible for the disease.

Gastroesophageal reflux disease (GERD). Researchers are investigating a possible link between idiopathic interstitial lung disease and gastroesophageal reflux disease. In GERD, stomach acid or occasionally bile salts enter the esophagus and then into the lungs.


Scar tissue formation in the lungs can lead to a range of life-threatening complications, including:

Low blood oxygen level (hypoxia)

Because interstitial lung disease reduces the amount of oxygen that enters the blood and lowers blood oxygen levels, there is a chance of having lower than normal blood oxygen levels. A severe lack of oxygen can disrupt basic body function.

Pulmonary hypertension

Unlike systemic hypertension, this condition affects only the arteries in the lungs. It begins when scar tissue constricts the tiniest blood vessels, restricting the blood flow in the lungs. This will increase the pressure in the pulmonary artery. Pulmonary hypertension is a serious disease that gradually gets worse.

Heart failure

This serious condition occurs when the right chamber of the heart is weaker - it has to pump harder than usual to move blood through a blocked pulmonary artery. Finally, the right ventricle is further impaired.

Respiratory failure

In the late stages of chronic interstitial lung disease, severe respiratory failure occurs when low blood oxygen levels coupled with increased pressure in the pulmonary arteries cause heart failure.

Tests and diagnostics

Identifying the disease and determining the cause of interstitial lung disease can be extremely difficult. The unusually large number of disorders of this type are very broad. Furthermore, the distinction between interstitial lung disease with identity and unknown causes is not always clear.

In addition, the signs and symptoms of a variety of medical problems - including chronic obstructive pulmonary disease (COPD), heart failure, and asthma - can resemble interstitial lung disease, and doctors must rule out them before making a definitive diagnosis.

To help cut down on confusion and rule out other possible illnesses, your doctor may start with a detailed medical history and physical exam. Once certain conditions have been ruled out, your doctor will recommend tests such as:


Although this is often the first test given in cases of suspected lung disease, radiography is not as effective as computed tomography (CT) in diagnosing interstitial lung disease. It can, however, help eliminate conditions that cause signs and symptoms similar to interstitial lung disease, including emphysema and lobar collapse.

High resolution computed tomography (HRCT) scan 

While traditional chest CT produces two-dimensional images of the lungs, CT scans use induction radiography and a computer to create cross-sectional images that add a lot of detail. HRCT shows lung tissue in great detail and provides more information than conventional CT.

Pulmonary function tests (PFTs)

Noninvasive tests are great for checking lung function. For the test, it usually requires blowing into an instrument called a spirometer, how much air the lungs can hold, and the flow of air out of the lungs. Scars get worse. Some lung function tests also measure the amount of gas exchanged across the membrane between the alveoli and capillaries.

Stress test

Because symptoms of interstitial lung disease are worse with activity, your doctor may evaluate lung function during exercise, usually on a stationary bike or treadmill. Although specific tests vary, blood pressure and blood oxygen levels are usually monitored with increased exercise.


In many cases, interstitial lung disease can be definitively diagnosed by examining a small amount of lung tissue (biopsy). During an endoscopic biopsy, the doctor inserts a bronchoscope through the mouth into the lung and takes one or more tissue samples, about the size of a needle tip. Then test in the lab. Bronchoscopy is performed on an outpatient basis using local anesthesia.

Bronchoalveolar lavage

In this procedure, the doctor injects saline through the bronchoscope and then immediately aspirates it. The recovered fluid contains cells from the alveoli.

Video to support surgery

When minimally invasive testing does not yield a specific diagnosis, a thoracic surgeon may perform a lung biopsy. During this procedure, a flexible tube with a camera (endoscope) is inserted through a small incision between the ribs, allowing the surgeon to view the lungs on a video monitor. Surgical instruments are then inserted through another incision, and the surgeon takes a sample of tissue in the lung.

Treatments and drugs

Interstitial lung disease caused by toxins or drugs is sometimes reversible when exposed to those substances is no longer possible. But in people who are not cases of progress, or little progress. Because some of the medications currently available can have serious side effects.

Treatment for interstitial lung disease usually includes a combination of the following drugs:


Anti-inflammatory drugs help a small number of people with interstitial lung disease. People are more likely to improve disorders with some cause and reverse changes in their lungs. Corticosteroids rarely improve lung function in people with idiopathic pulmonary fibrosis, and these benefits are usually temporary.

Taking the drug for a long time or with large doses of corticosteroids can cause a number of side effects, including glaucoma, osteoporosis, increased blood sugar leading to diabetes, delayed wound healing, and increased mobility. susceptible to infection.


This cytotoxic drug, commonly used to prevent organ rejection after transplantation, may also be used in combination with corticosteroids to treat interstitial lung disease. Azathioprine can cause serious side effects, including a decrease in blood cells and an increased risk of infection and cancer. A cytotoxic drug, cyclophosphamide can be used if azathioprine is not effective. However, the side effects of cyclophosphamide are even more severe.


This medication belongs to a group of drugs called antioxidants. Oxidation is a natural process that leads to cell and tissue damage. The process may play a role in the development of scarring in the lungs (pulmonary fibrosis). Results from a clinical trial showed that combining corticosteroids with acetylcysteine ​​and azathioprine improved lung function in people with idiopathic pulmonary fibrosis. No significant changes were seen in mortality.


These medications are sometimes used to help reduce the growth of scar tissue. In clinical studies, the drugs bosentan and pirfenidone have shown promise in slowing the progression of lung injury. Follow-up studies to get information about the risks and benefits of these drugs in the treatment of interstitial lung disease are ongoing. Other anti-fibrotic drugs that have been studied as possible treatments for the condition - including colchicine, penicillamine, and interferon gamma-1b - have not been found effective.

Oxygen therapy

Depending on the severity of your symptoms and activity level, your doctor may recommend oxygen therapy. Although oxygen cannot stop lung damage, it can make breathing and exercising easier and prevent or reduce complications caused by low blood oxygen. Oxygen therapy can also improve sleep. It can also lower right heart chamber pressure.

Pulmonary rehabilitation

This is a formal program for people with chronic lung disease including medical management. The purpose of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning but also to help people with pulmonary fibrosis live full lives. Pulmonary rehabilitation programs focus on exercise, instruction in breathing more efficiently, education and emotional support, and nutritional counseling.

Typically, a multifaceted approach requires a team of health care providers that may include doctors, nurses, rehabilitation specialists, dietitians, and social workers. Programs can be very different, however.

Lung transplant

This may be an option for people with severe interstitial lung disease who are unlikely to benefit from other treatment options.

To be considered for a transplant, you must agree to quit if you smoke, be healthy enough to undergo surgery and post-transplant treatment and be willing and able to follow the medical plans put forth by your rehabilitation team. rehab and transplant, patience and mental strength, and support to go through the wait for a donor agency.

Lifestyle and remedies

Being involved in treatment and being as healthy as possible is essential to living with interstitial lung disease. Take the following steps to benefit your health:

Stop smoking. There is a link between smoking and certain types of idiopathic interstitial lung disease, the most serious and potentially deadly form of the disorder. Talk to your doctor about options for stopping smoking, including smoking cessation programs, which use a variety of proven techniques to help people quit.

Enroll in a pulmonary rehabilitation program. These programs vary widely, but generally they focus on improving the ability to exercise and perform normal activities, manage dyspnea with breathing techniques, and improve appetite and well-being. nutrition, exposure to the difficult psychological aspects of living with lung disease, and improving the overall quality of life.

Exercise regularly as prescribed by your doctor. Exercise is a double-edged sword for people with lung disease, it requires an increased amount of oxygen, and it makes symptoms worse. At the same time, exercise is essential for maintaining lung function, reducing stress and depression, and maintaining overall health and well-being. Ask your doctor for a referral to a pulmonary rehabilitation program before starting or continuing one.

Eat well. People with lung disease can lose weight because it's uncomfortable to eat and because of the extra energy it takes to breathe. However, a nutrient-rich diet contains the necessary amount of calories. The types of foods eaten, the time of day eaten and the size of portions can all play a role in nourishment.

Because an empty stomach usually breathes easier, it is possible to eat several small meals throughout the day instead of two or three large meals. You can also try choosing lighter foods, such as fruits and vegetables, rather than high-fat foods, which take more energy to digest. A dietitian can provide additional guidance for eating.

If you're overweight, losing weight to achieve a healthy body mass index (BMI) can have a huge effect on your ability to breathe and exercise.

Coping and supporting

Living with chronic lung disease is emotionally and physically challenging. Daily routines and activities may need to be adjusted, sometimes completely, as breathing problems get worse or health care needs to be prioritized in life. Feelings of anger, fear, and sadness are normal when grieving a loss and worrying about what's next for you and your family.

These suggestions may help cope with interstitial lung disease:

Share your feelings with loved ones and doctors. Talking openly can help and help loved ones cope with the challenging emotions of illness. In addition, clear communication helps and effective family planning for needs if the disease progresses.

Consider joining a support group. In the support group, will be together with people who are facing similar challenges. Group members can share coping strategies, exchange information about new treatments, or simply listen when expressing feelings.