Pulmonary Hypertension in Congenital Heart Disease: A Scientific Statement From the American Heart Association

Interstitial Lung Disease (ILD): Symptoms, Causes, Diagnosis ... - WebMD

ILD symptoms may include shortness of breath, dry cough, weight loss, fatigue, and chest discomfort. (Photo Credit: E+/Getty Images)

Interstitial lung disease (ILD) is a group of many lung conditions. All interstitial lung diseases affect the interstitium, a part of your lungs.

The interstitium is a lace-like network of tissue that goes throughout both lungs. It supports your lungs' tiny air sacs, called alveoli. Normally, the interstitium is so thin that it doesn't show up on X-rays or CT scans.

Interstitial lung infections scar and inflame your lungs, leading to shortness of breath and other serious problems.

All forms of ILD cause the interstitium to thicken. This can happen from inflammation, scarring, or a buildup of fluid. Some forms of ILD last a short time (acute), while others are long-term (chronic) and don't go away.

Some types of interstitial lung disorders include:

Interstitial pneumonia. Bacteria, viruses, or fungi can infect your interstitium. A bacteria called Mycoplasma pneumoniae is the most common cause.

Idiopathic pulmonary fibrosis. This makes scar tissue grow in your interstitium. The word "idiopathic" means its cause is unknown.

Nonspecific interstitial pneumonitis. This is an ILD that often affects people with autoimmune conditions such as rheumatoid arthritis or scleroderma.

Hypersensitivity pneumonitis. This happens when dust, mold, or other things that you breathe irritate your lungs over a long time.

Cryptogenic organizing pneumonia (COP). COP is a pneumonia-like ILD without an infection. You might hear your doctor call this bronchiolitis obliterans with organizing pneumonia (BOOP).

Acute interstitial pneumonitis. This is a sudden, severe form of ILD. People who have it often need to be connected to a machine called a ventilator that breathes for them.

Desquamative interstitial pneumonitis. This is an ILD that partly results from smoking.

Sarcoidosis. This causes ILD along with swollen lymph nodes. It can also affect your heart, skin, nerves, and eyes.

Asbestosis. This is an ILD caused by breathing asbestos, a fiber used in building materials.

The most common symptom of all forms of ILD is shortness of breath, either generally or after some physical activity. Almost everyone with ILD will have breathlessness, which can worsen over time.

Other symptoms of ILD include:

Cough, which is usually dry and doesn't bring up mucus

Weight loss, most often in people with COP or BOOP

Fatigue

Chest discomfort

With most forms of ILD, shortness of breath develops slowly (over months). But if you have interstitial pneumonia or acute interstitial pneumonitis, your symptoms will come on quickly (in hours or days).

Bacteria, viruses, and fungi can cause interstitial pneumonia. You can also get ILD if you regularly breathe in things that can damage your lungs. These include:

Asbestos

Bird proteins (from animals such as exotic birds, chickens, or pigeons)

Coal dust or various other metal dusts from working in mining

Grain dust from farming

Silica dust

Talc

It's rare, but certain drugs can cause ILD. They include:

Some antibiotics, such as nitrofurantoin (Macrobid, Macrodantin, Nitro Macro)

Some anti-inflammatory drugs, such as rituximab (Riabni, Rituxan, Ruxience, Truxima)

Chemotherapy drugs, such as bleomycin (Blenoxane, Bleo 15k)

Heart medications, such as amiodarone (Cordarone, Pacerone)

However, the cause of most ILDs is unknown.

Jeremy Katzen, MD, assistant professor of medicine (pulmonary, allergy, and critical care) at Penn Medicine says that although we know what can trigger ILD, we don't fully understand why some people with conditions like rheumatoid arthritis or those exposed to bird proteins develop ILD, while others don't. Scientists are still trying to figure out the basic causes of the disease at the genetic and cellular levels. This research is important because it'll help us understand why some people are more likely to get ILD than others.

Anyone can get ILD, but some things can increase your risk:

Age. Adults, particularly those over 70, are much more likely to get ILD than children. But kids can get it, too.

Autoimmune disease.Lupus, rheumatoid arthritis, and scleroderma can raise your risk for ILD.

Gastroesophageal reflux disease (GERD). This condition can also put you at higher risk.

Genetics. Some conditions are passed down among family members.

History of certain diseases. This includes things such as hepatitis C, tuberculosis, pneumonia, and chronic obstructive pulmonary disease (COPD).

Smoking. This habit can increase your risk of developing ILD.

Sarcoidosis. This is a disease where you grow groups of tiny inflammatory cells (granulomas) in any part of your body, but most often in your lungs or lymph nodes.

Radiation treatments. Cancer treatments may raise your risk for ILD.

Work environment. If you breathe in coal dust or asbestos at work, this can raise your risk.

People with ILD usually go to the doctor because of shortness of breath or a cough. Your doctor will probably use imaging tests of your lungs to find out the problem.

Chest X-ray. A simple chest X-ray is the first test for most people with a breathing problem. If you have ILD, chest X-rays may show fine lines in your lungs.

CT scan. A CT scan takes multiple X-rays of your chest and uses a computer to create detailed images of your lungs and surrounding structures. These tests can usually find ILD.

High-resolution CT scan. If your doctor thinks you have ILD, certain CT scan settings can get better-quality images of your interstitium. This can help your doctor diagnose you.

Lung function test. For this test, you sit in a sealed plastic booth and breathe through a tube. It measures your total lung capacity and your ability to transfer oxygen from your lungs to blood, both of which might be lowered if you have ILD.

Lung biopsy. Looking at lung tissue under a microscope is often the only way to find out the type of ILD you have. Lung tissue is collected in a procedure called a lung biopsy, which can be done in several ways:

A bronchoscopy is where the doctor will run a tube called a bronchoscope through your mouth or nose into your airways. Tiny tools on the bronchoscope can take a sample of lung tissue.

Video-assisted thoracoscopic surgery (VATS) is a process where your doctor will make small cuts to insert tools that take samples from multiple areas of lung tissue.

Open lung biopsy (thoracotomy) may be needed in some cases. This surgery involves a large incision (cut) in your chest to get a lung biopsy.

ILD doesn't have stages, but your condition may be classified as mild, moderate, or severe. 

The terms mild, moderate, and severe describe how well your lungs are working based on tests called pulmonary function tests. "These tests measure the size of the lungs (vital capacity) as well as the ability to absorb oxygen from the air (diffusing capacity)," says Sonye Danoff, MD, professor of medicine at Johns Hopkins Medicine and director of the Johns Hopkins Interstitial Lung Disease/Pulmonary Fibrosis Program. 

The most common symptoms of ILD are shortness of breath and a cough. People with mild lung damage might not notice shortness of breath unless they're exercising intensely, she says. But as your lung damage becomes severe, you may notice shortness of breath with less activity and even while at rest. A nagging, dry cough is also common in ILD and can happen at any stage. But it tends to get worse and more bothersome as the condition worsens. 

There's no cure for ILD. Treatment depends on the type you have and its cause. Here are some treatment options:

Inhaled oxygen. If you have low oxygen levels because of ILD, inhaled oxygen may help your symptoms. Regular use of oxygen might also protect your heart from damage caused by low oxygen levels.

Physical therapy. Your therapist can show you exercises and breathing techniques that may improve your lung capacity.

Lung transplant. In advanced ILD that has severely affected you, you may need a lung transplant. Most people who have a lung transplant for ILD see major improvements in their quality of life and ability to exercise.

ILD medications

Antibiotics. These treat most cases of interstitial pneumonia. Types of pneumonia caused by a virus usually get better on their own. The types caused by fungi are rare and can be treated with antifungal drugs.

Corticosteroids. In some forms of ILD, inflammation in your lungs causes damage and scarring. Corticosteroids can slow down your immune system. This lessens the amount of inflammation in your lungs and the rest of your body.

N-acetylcysteine (Mucomyst). This potent antioxidant may slow the decline of lung function in some forms of ILD. You'll take it in combination with other treatments.

Drugs that are considered controversial for ILD treatment include:

Cyclophosphamide (Cytoxan)

Cyclosporine

Methotrexate

Nintedanib (Ofev)

Pirfenidone (Esbriet)

These medicines affect the way your immune system works. If your doctor thinks you need one, they'll keep a close watch on you while you're taking it. These medications can have serious side effects.

Since ILD can't be cured, it's important to take care of yourself to minimize the effects. 

Treat any diseases (such as GERD) that might be contributing to your ILD.

If you work in an environment where you can inhale coal dust or asbestos, be sure to wear a respirator (a mask that filters out air particles). Try to find a job where you don't get exposed to these types of dust.

Wear a respirator around things like hay, bird droppings, or HVAC systems if they cause you to have severe allergic reactions.

Quit smoking if you're a smoker and avoid secondhand smoke.

Eat properly. With lung diseases, you may not feel like eating as much as you regularly would. Plus, you might lose weight because of the extra energy involved in breathing.

Get vaccinated against pneumonia and the flu. Respiratory infections can worsen ILD symptoms. 

Stay active. "I tell patients that diseases that make people feel short of breath, like ILD, can shrink people's physical worlds. If it's uncomfortable to go for a walk around the block because of dyspnea (shortness of breath), it is easy (and natural) to stop going for those walks to avoid that discomfort," says Katzen. "The issue with this is that it can cause deconditioning, which makes the dyspnea with these activities more severe. Staying active, discussing with your ILD provider whether pulmonary rehabilitation is an appropriate option, and addressing cardiovascular health, are all ways to avoid a cycle of dyspnea and deconditioning."

Severe ILD can cause life-threatening complications. These include:

Pulmonary hypertension. This is high blood pressure in your lungs. Low levels of oxygen or scar tissue can narrow tiny blood vessels in your lungs and reduce blood flow. This can raise the pressure in your lung's arteries and make it harder for oxygen to get into your blood. Over time, your right ventricle (lower right chamber of your heart) may weaken or fail because of this pressure.

Right-sided heart failure (cor pulmonale). This happens when your right ventricle — responsible for pumping blood to your lungs — has to work harder due to blocked arteries. Over time, this extra work can cause the heart to fail. This is often a result of pulmonary hypertension.

Respiratory failure. In the late stage of chronic ILD, very low blood oxygen levels and increased pressure in your lung's arteries can cause your heart to fail.

Collapsed lung (pneumothorax). Air builds up in the space between your chest wall and lung, pressing against the lung. This causes your lung to collapse, either partially or completely.

Lung cancer. This type of cancer is also a complication of severe ILD. If you have severe ILD, your risk of developing lung cancer is higher.

Here are some ways to avoid getting ILD:

Stay away from toxins such as asbestos, coal dust, and silica dust, in your work area. If you must be around them, wear a respirator.

Stay away from toxins in the environment, such as bird droppings, feathers, mold, and bacteria. Wearing a respirator can help if you can't avoid these.

Don't smoke and avoid secondhand smoke.

If you have a medical condition (hepatitis C, pneumonia, COPD, or sarcoidosis) that puts you at a higher risk for ILD, ask your doctor about what you can do to prevent this from happening.

Interstitial lung disease (ILD) is a group of medical conditions that scar your lungs and make it harder for you to breathe. These include interstitial pneumonia and idiopathic pulmonary fibrosis. Breathing in toxic fumes from coal dust, asbestos, or mold can cause ILD, and so can having an autoimmune disease. Often, you may have ILD without knowing the reason. There's no known cure, but you can manage your condition with medication and physical therapy.

What's the life expectancy of someone with interstitial lung disease?

It depends on the severity of the disease. A mild condition may not shorten your life. In severe cases of ILD, life expectancy may be about three to five years after diagnosis.

Can you recover from interstitial lung disease?

Once your lungs are scarred, this can't be reversed. But you can have treatment to help you live with ILD. This would include medication, physical therapy, inhaled oxygen, and in very severe cases, a lung transplant.

What's the most common cause of death in interstitial lung disease?

The most common cause of death in ILD is idiopathic pulmonary fibrosis.

How do I know if I have interstitial lung disease?

If you have breathing problems (shortness of breath or a dry cough), see your doctor. Breathing problems don't always mean you have ILD, but they're an early sign of it.

Types Of Lung Diseases & Their Causes - WebMD

Lung diseases are some of the most common medical conditions in the world. Tens of millions of people have lung disease in the U.S. Alone. Smoking, infections, and genes cause most lung diseases.

Your lungs are part of a complex system, expanding and relaxing thousands of times each day to bring in oxygen and send out carbon dioxide. Lung disease can happen when there are problems in any part of this system.

Your windpipe (trachea) branches into tubes called bronchi, which in turn become smaller tubes throughout your lungs. Diseases that can affect these airways include:

Asthma. Your airways are constantly inflamed and may spasm, causing wheezing and shortness of breath. Allergies, infections, or pollution can trigger asthma symptoms.

Chronic obstructive pulmonary disease (COPD). With this lung condition, you can't exhale the way you usually would, which causes trouble breathing.

Chronic bronchitis. This form of COPD brings a long-term wet cough.

Emphysema. Lung damage allows air to be trapped in your lungs in this form of COPD. Trouble blowing air out is its hallmark.

Acute bronchitis. This sudden infection of your airways is usually caused by a virus.

Cystic fibrosis. With this condition, you have trouble clearing mucus out of your bronchi. This leads to repeated lung infections.

Your airways branch into tiny tubes (bronchioles) that end in clusters of air sacs called alveoli. These air sacs make up most of your lung tissue. Lung diseases affecting your alveoli include:

Pneumonia. An infection of your alveoli, usually by bacteria or viruses, including the coronavirus that causes COVID-19.

Tuberculosis Pneumonia that slowly gets worse, caused by the bacteria Mycobacterium tuberculosis.

Emphysema. This happens when the fragile links between alveoli are damaged. Smoking is the usual cause. (Emphysema also limits airflow, affecting your airways.)

Pulmonary edema. Fluid leaks out of the small blood vessels of your lung into the air sacs and the area around them. One form is caused by heart failure and back pressure in your lungs' blood vessels. In another form, injury to your lung causes the leak of fluid.

Lung cancer. It has many forms and may start in any part of your lungs. It most often happens in the main part of your lung, in or near the air sacs.

Acute respiratory distress syndrome (ARDS). This is a severe, sudden injury to the lungs from a serious illness. COVID-19 is one example. Many people who have ARDS need help breathing from a machine called a ventilator until their lungs recover.

Pneumoconiosis. This is a category of conditions caused by inhaling something that injures your lungs. Examples include black lung disease from coal dust and asbestosis from asbestos dust.

The interstitium is the thin, delicate lining between your alveoli. Tiny blood vessels run through the interstitium and let gas transfer between the alveoli and your blood. Various lung diseases affect the interstitium:

The right side of your heart gets low-oxygen blood from your veins. It pumps blood into your lungs through the pulmonary arteries. These blood vessels can have diseases, as well.

Pulmonary embolism (PE). A blood clot (usually in a deep leg vein, called deep vein thrombosis) breaks off, travels to your heart, and gets pumped into your lungs. The clot sticks in a pulmonary artery, often causing shortness of breath and low blood oxygen levels.

Pulmonary hypertension. Many conditions can cause high blood pressure in your pulmonary arteries. This can lead to shortness of breath and chest pain. If your doctor can't find a cause, they'll call it idiopathic pulmonary arterial hypertension.

The pleura is the thin lining that surrounds your lung and lines the inside of your chest wall. A tiny layer of fluid lets the pleura on your lung's surface slide along the chest wall with each breath. Lung diseases of the pleura include:

Pleural effusion. Fluid collects in the space between your lung and the chest wall. Pneumonia or heart failure usually causes this. Large pleural effusions can make it hard to breathe and may need to be drained.

Pneumothorax. Air may get into the space between your chest wall and the lung, collapsing the lung.

Mesothelioma. This is a rare form of cancer that forms on the pleura. Mesothelioma tends to happen several decades after you come into contact with asbestos.

Your chest wall also plays an important role in breathing. Muscles connect your ribs to each other, helping your chest expand. Your diaphragm descends with each breath, also causing chest expansion. Diseases that affect your chest wall include:

Understanding Post-COVID Interstitial Lung Disease: Causes, Treatment ...

After being hospitalized for three months with COVID-19 and double pneumonia in 2020, Houston police officer Hito Bazan awoke one morning to discover that he couldn't talk, eat, walk, or most importantly, breathe. Bazan was diagnosed with interstitial lung disease (ILD) known as pulmonary fibrosis (PF).

Three years into the COVID-19 pandemic, we are beginning to see the potential long-term effects of COVID-19, including, in some select cases, PF and ILD. As a relatively unknown disease, it is important to understand how post-COVID ILD occurs, and share the resources and treatment options available for those, like Bazan, who are affected by this lung condition.

"There weren't a lot of answers in the beginning," Bazan said. "I've come a long way since then, and we know a lot more about post-COVID ILD than we did nearly three years ago."

What is Post-COVID ILD?

In its simplest sense, PF and ILD refers to scarring in the lung tissue. Over time, scar tissue may build up and block the movement of oxygen from the lungs into the bloodstream. Low oxygen levels – and the stiff scar tissue itself – can cause people who have PF or ILD to feel short of breath, particularly when walking and exercising.

Respiratory symptoms of ILD after COVID-19 may be similar to those of other types of ILD. Common symptoms of ILD include a chronic, dry cough, fatigue, and shortness of breath. While some people may have worsening symptoms over time, some patients can have gradual improvement in symptoms, and others can have stable but persistent symptoms. People with ILD after COVID-19 may experience additional symptoms of Post-Acute Sequelae of COVID-19 (PASC) or "long COVID," including heart palpitations, chest pain, or difficulty thinking and concentrating.

The symptoms of shortness of breath and cough are common to many different illnesses, so if one has these symptoms following a COVID-19 infection, it does not always mean that ILD is present. Persistent symptoms should always prompt a conversation with your medical provider."

Dr. Amy Hajari Case, Senior Medical Advisor for Education and Awareness for the Pulmonary Fibrosis Foundation (PFF)

Causes of ILD after COVID-19

Because COVID-19 pneumonia causes inflammation in the lungs, Bazan was at the highest risk for developing PF and ILD. People with severe cases of COVID-19 infection – particularly those who develop acute respiratory distress syndrome (ARDS) and require mechanical ventilation – are also at risk to develop the disease. In many cases, the lung damage from ARDS will fully resolve over time, but in other cases, permanent lung damage can develop.

The ways in which less severe COVID-19 infection causes ILD and PF are still unclear. Studies are ongoing to better understand this connection and the risk factors for development of ILD after COVID-19. These studies aim to determine if ILD seen in patients who have had COVID-19 is inflammation that improves with time, if it is fibrosis that does not improve but is stable, or if it is a disease that continues to worsen over time.

"The impact of the COVID-19 pandemic has been unprecedented. We are still learning the ways in which post-COVID ILD affects patients and continue to gain a better understanding of the progression of the disease for patients with different risk factors and exposures," added Dr. Hajari Case. "In time, we are hopeful that more research will emerge to shed light on the causes of post-COVID ILD and the long-term effects on our overall health."

Diagnosing post-COVID ILD

People who develop pneumonia from COVID-19 will often have chest imaging, such as a chest x-ray or CT scan, as part of their medical evaluation. Abnormalities found on chest imaging or persistent respiratory symptoms after recovery from a COVID-19 infection can be clues that ILD may be present.

A healthcare provider who suspects that a patient has ILD might also perform blood work and pulmonary function tests to measure how much air the lungs can hold. Doctors may also perform a high-resolution computed tomography (HRCT) scan, which shows detailed images of the lung. In some cases, a diagnosis of ILD can be made from these tests and in others, a lung biopsy may need to be performed. Patients may be monitored over time to evaluate if the scarring or inflammation progresses, stabilizes, or resolves. Following his own diagnosis with PF, Bazan has had to make several life changes, including retiring from his career as a police officer. However, through finding the right medical care, resources and the use of an oxygen machine, he has been able to exercise, complete DIY projects around his home, and spend time with his family.

"It was a long road getting back to where I am now," Bazan said. "I used to be very focused on work, but now I'm driven to beat this disease, and I'm not going to stop living. I've learned that life does not end – it just takes some adjustments."

Treating post-COVID ILD

Currently, there are no guidelines or clinical trials available to help clinicians make treatment decisions. Monitoring a person's disease without medication is appropriate in some cases. In other cases, when inflammation is present in the lung, steroid medications, such as prednisone, are commonly used. The antifibrotic medications pirfenidone and nintedanib, which can be prescribed to treat other forms of PF, are currently being studied in clinical trials of ILD after COVID-19.

"While there is significant uncertainty regarding the prognosis of ILD after COVID-19, studies show that most survivors of severe illness from COVID-19 experience gradual improvement or stability, although they may have ongoing lung function impairment if they developed PF," concluded Dr. Hajari Case. "Studies are essential to better understand the natural history and risk factors for development of ILD after COVID-19."

Despite not having a cure for the disease, post-COVID ILD patients like Bazan can find support and resources to improve their quality of life through the Pulmonary Fibrosis Foundation (PFF).

"What helps the most is talking to people who have gone through similar struggles and can show you what is possible on this journey," Bazan said. "At the PFF, you'll find a strong community of people with the disease and those who are engaged in the fight against PF."

The PFF provides education and resources for those affected by post-COVID ILD. Pulmonary rehabilitation, supplemental oxygen, smoking cessation, routine vaccinations (such as influenza, COVID-19, and pneumonia vaccination), and disease management by a specialist skilled in treating patients who have ILD can improve quality of life while living with ILD. Patients experiencing post-COVID ILD are also encouraged to search for research studies via the PFF Clinical Trial Finder and utilize the PFF Care Center Network to connect with a community of patients and support groups affected by the disease.

"Two years ago, the quote 'breathing is the greatest pleasure in life' wouldn't have meant much to me. Today, it means everything," Bazan said. "With each breath, I'm determined not to let this disease keep me down. I'm going to continue living my life the best I can."

To learn more about PF and ILD, explore treatment options or seek support for post-COVID ILD, review the PFF's Post COVID-19 Interstitial Lung Disease fact sheet and visit pulmonaryfibrosis.Org.

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