Pulmonary Embolism

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Pulmonary embolism, or PE, is a sudden blockage in a lung artery. The blockage usually is caused by a blood clot that travels to the lung from a vein in the leg.

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A clot that forms in one part of the body and travels in the bloodstream to another part of the body is called an embolus (EM-bo-lus).

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PE is a serious condition that can:

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• Damage part of your lung because of a lack of blood flow to your lung tissue. This damage may lead to pulmonary hypertension (increased pressure in the pulmonary arteries).

• Cause low oxygen levels in your blood.

• Damage other organs in your body because of a lack of oxygen.

 

If a blood clot is large, or if there are many clots, PE can cause death.

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PE most often is a complication of a condition called deep vein thrombosis (DVT). In DVT, blood clots form in the deep veins of the body—most often in the legs

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Interstitial Lung Disease

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Interstitial lung disease (ILD) is a group of lung disorders in which the lung tissues become inflamed and then damaged.

 

The lungs contain tiny air sacs (alveoli), which is where oxygen is absorbed. These air sacs expand with each breath.

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The tissue around these air sacs is called the interstitium. In people with interstitial lung disease, this tissue becomes stiff or scarred, and the air sacs are not able to expand as much. As a result, not as much oxygen can get to the body.

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ILD can occur without a known cause. This is called idiopathic ILD. Idiopathic pulmonary fibrosis (IPF) is the most common disease of this type.

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There are also dozens of known causes of ILD, including:

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• Autoimmune diseases (in which the immune system attacks the body) such as lupus, rheumatoid arthritis, sarcoidosis, and scleroderma

• Lung inflammation due to breathing in a foreign substance such as certain types of dust, fungus, or mold (hypersensitivity pneumonitis)

• Medicines (such as nitrofurantoin, sulfonamides, bleomycin, amiodarone, methotrexate, gold, infliximab, and etanercept)

• Radiation treatment to the chest

• Working with or around asbestos, coal dust, cotton dust, and silica dust (called occupational lung disease) 

 

Cigarette smoking may increase the risk of developing some forms of ILD and may cause the disease to be more severe.

 

Shortness of breath is a main symptom of ILD. You may breathe faster or need to take deep breaths:

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• At first, shortness of breath may not be severe and is only noticed with exercise, climbing stairs, and other activities.

• Over time, it can occur with less strenuous activity such as bathing or dressing, and as the disease worsens, even with eating or talking. 

 

Most people with this condition also have a dry cough. A dry cough means you do not cough up any mucus or sputum.

 

Over time, weight loss, fatigue, and muscle and joint pain are also present.

 

People with more advanced ILD may have:

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• Abnormal enlargement of the base of the fingernails (clubbing)

• Blue color of the lips, skin, or fingernails due to low blood oxygen levels (cyanosis) 

• Symptoms of the other diseases such as arthritis or trouble swallowing (scleroderma), associated with ILD

 

The health care provider will perform a physical exam. Dry, crackling breath sounds may be heard when listening to the chest with a stethoscope. 

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The following tests may be done:

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• Blood tests to check for autoimmune diseases

• Bronchoscopy with or without biopsy

• Chest x-ray

• High resolution CT (HRCT) scan of the chest

• MRI chest

• Echocardiogram

• Open lung biopsy

• Measurement of the blood oxygen level at rest or when active

• Blood gases

• Pulmonary function tests

• Six minute walk test (checks how far you can walk in 6 minutes and how many times you need to stop to catch your breath) 

 

People who are heavily exposed to known causes of lung disease in the workplace are usually routinely screened for lung disease. These jobs include coal mining, sand blasting, and working on a ship.

 

Treatment depends on the cause and duration of the disease. Medicines that suppress the immune system and reduce swelling in the lungs are prescribed if an autoimmune disease is causing the problem. For some people who have IPF, pirfenidone and nintedanib are two medicines that may be used to slow the disease. If there is no specific treatment for the condition, the aim is to make you more comfortable and support lung function:

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• If you smoke, ask your provider about how to stop smoking.

• People with low blood oxygen levels will receive oxygen therapy in their home. A respiratory therapist will help you set up the oxygen. Families need to learn proper oxygen storage and safety. 

Lung rehabilitation can provide support, and help you learn:

• Different breathing methods

• How to set up your home to save energy

• How to eat enough calories and nutrients

• How to stay active and strong 

Some people with advanced ILD may need a lung transplant.

 

You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.

 

The chance of recovering or ILD getting worse depends on the cause and how severe the disease was when it was first diagnosed.

Some people with ILD develop heart failure and high blood pressure in the blood vessels of their lungs.

 

Idiopathic pulmonary fibrosis has a poor outlook.

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Pulmonary Hypertension

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Pulmonary hypertension, or PH, is increased pressure in the pulmonary arteries. These arteries carry blood from your heart to your lungs to pick up oxygen.

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PH causes symptoms such as shortness of breath during routine activity (for example, climbing two flights of stairs), tiredness, chest pain, and a racing heartbeat. As the condition worsens, its symptoms may limit all physical activity.

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To understand PH, it helps to understand how your heart and lungs work. Your heart has two sides, separated by an inner wall called the septum.

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Each side of your heart has an upper and lower chamber. The lower right chamber of your heart, the right ventricle, pumps blood to your pulmonary arteries. The blood then travels to your lungs, where it picks up oxygen.

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The upper left chamber of your heart, the left atrium, receives the oxygen-rich blood from your lungs. The blood is then pumped into the lower left chamber of your heart, the left ventricle. From the left ventricle, the blood is pumped to the rest of your body through an artery called the aorta.

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PH begins with inflammation and changes in the cells that line your pulmonary arteries. Other factors also can affect the pulmonary arteries and cause PH. For example, the condition may develop if:

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• The walls of the arteries tighten.

• The walls of the arteries are stiff at birth or become stiff from an overgrowth of cells.

• Blood clots form in the arteries.

 

These changes make it hard for your heart to push blood through your pulmonary arteries and into your lungs. As a result, the pressure in your arteries rises. Also, because your heart is working harder than normal, your right ventricle becomes strained and weak.

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Your heart may become so weak that it can't pump enough blood to your lungs. This causes heart failure. Heart failure is the most common cause of death in people who have PH.

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PH is divided into five groups based on its causes. In all groups, the average pressure in the pulmonary arteries is higher than 25 mmHg at rest or 30 mmHg during physical activity. The pressure in normal pulmonary arteries is 8–20 mmHg at rest. (The mmHg is millimeters of mercury—the units used to measure blood pressure.)

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Other diseases or conditions, such as heart and lung diseases or blood clots, usually cause PH. Some people inherit the condition (that is, their parents pass the genes for PH on to them). In some cases, the cause isn't known.

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PH has no cure. However, research for new treatments is ongoing. The earlier PH is treated, the easier it is to control.

Treatments include medicines, procedures, and other therapies. These treatments can relieve PH symptoms and slow the progress of the disease. Lifestyle changes also can help control symptoms.

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Sarcoidosis

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Sarcoidosis is a condition that develops when groups of cells in your immune system form red and swollen (inflamed) lumps, called granulomas, in various organs in the body. The inflammation that leads to these granulomas can be caused either by infections or by certain things you come into contact with in your environment.

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Sarcoidosis can affect any organ. Most often it affects the lungs and lymph nodes in the chest. You may feel extremely tired or have a fever. You may also have other symptoms depending on what organ is affected. Your doctor will diagnose sarcoidosis in part by ruling out other diseases that have similar symptoms.

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Not everyone needs treatment. Treatment will depend on your symptoms and which organs are affected. Medicines can help treat the inflammation or lower your body’s immune response. Many people recover with few or no long-term problems. Sometimes the disease causes permanent scarring (fibrosis) in the lungs or other organs and can lead to life-threatening heart or lung problems.

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Many people who have sarcoidosis have no symptoms, or they may feel unwell but without any obvious symptoms. Others may be depressed, feel very tired, or have a general feeling of discomfort. You may also faint or have unexplained weight loss.

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Lofgren’s syndrome

Lofgren’s syndrome is a classic set of symptoms of sarcoidosis that includes:

• Swollen lymph nodes in your chest, neck, chin, armpits, or groin

• A rash of small, itchy, or painful bumps called erythema nodosum that most commonly appear on your head, neck, or legs

• Blurred vision, eye pain or redness, light sensitivity, or watery eyes

• Joint pain, stiffness, or swelling

• Fever

 

Some people have Lofgren’s syndrome when they first develop sarcoidosis. This is most common in women between ages 30 and 40. It usually goes away completely within 2 years.

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Sarcoidosis in the lungs

If you have sarcoidosis in the lungs, you may experience these symptoms:

• Wheezing

• Coughing

• Shortness of breath

• Chest pain

 

However, you can have sarcoidosis in the lungs without these symptoms. For example, skin rashes or sores can include erythema nodosum (explained above) or lupus pernio. Lupus pernio causes skin sores that usually affect the face, especially the nose, cheeks, lips, and ears. These sores usually last a long time. Lupus pernio affects African American people more often than other groups.

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Other symptoms

You may have other symptoms based on which organs are affected:

• Larger than normal liver or spleen or jaundice, which can make your eyes or skin yellow

• Nervous system problems, such as headache, dizziness, vision problems, seizures, mood swings, hallucinations, delusions, or nerve pain

• Heart palpitations or an irregular heartbeat

• Abdominal pain, nausea, or vomiting

• Muscle pain or soreness

• Swollen salivary glands

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Diagnosis of Sarcoidosis

Sarcoidosis is diagnosed based on your symptoms, a physical exam, and imaging tests or a biopsy. Before diagnosing you with sarcoidosis, your healthcare provider will rule out other possible conditions.

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Stages of sarcoidosis

Doctors use stages to describe sarcoidosis of the lung or lymph nodes of the chest. The stages are based on where the lumps, or granulomas, are found and whether there is scarring on imaging tests. Stage IV is the most serious and means you have permanent scarring in the lungs.

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Medical history and physical exam

Bring a list of symptoms with you to your appointment. Tell your provider if you live or work near insecticides or mold or have other risk factors for sarcoidosis. During the physical exam, your provider may:

• Check your temperature.

• Check to see if your lymph nodes, spleen, or liver are swollen.

• Listen to your chest with a stethoscope as you breathe in and out.

• Look for rashes or sores on your body, such as scalp and lower legs.

 

Diagnostic tests and procedures

There are no screening tests to determine who will develop sarcoidosis. If you are at risk for sarcoidosis, your provider may talk to you about trying to avoid certain substances in your environment that can trigger granulomas.

Your healthcare provider may have you undergo certain tests and procedures to diagnose sarcoidosis.

• Chest X-rays look for granulomas or scarring in the lungs and heart. This will also help figure out the stage of the disease. Often, sarcoidosis is found because a chest X-ray is done for another reason.

• biopsy of the skin, lymph nodes, lungs, or other affected organs may help confirm your sarcoidosis diagnosis. Your doctor will do a bronchoscopy to get the biopsy sample from your lungs or lymph nodes in your chest.

• Blood tests check your blood counts, hormone levels, and how well your kidneys are working.

• Other imaging tests look for granulomas or inflammation in the heart, eyes, lymph nodes, or other areas. These may include magnetic resonance imaging (MRI) or an ultrasound.

The tests below look at how sarcoidosis is affecting the body.

• Neurological tests, such as electromyography, evoked potentials, spinal taps, or nerve conduction tests, look for problems with the nervous system caused by sarcoidosis.

• Eye exams look for eye damage, which can occur without symptoms in a person with sarcoidosis.

• Lung function tests check whether you have breathing problems.

• Heart tests monitor how well your heart is working. Sarcoidosis only rarely affects the heart, but cardiac sarcoidosis may be life threatening. Tests may include electrocardiography (ECG or EKG), echocardiography, or cardiac MRI.

 

What causes sarcoidosis?

Your immune system creates inflammation to help defend you against germs and sickness. But in sarcoidosis, inflammation goes off track and the cells in your immune system form lumps, called granulomas, in your body. Over time, inflammation may lead to permanent scarring of organs.

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Studies suggest that some immune system triggers can lead to sarcoidosis in certain people. Triggers can include infections without symptoms or coming into contact with substances in the environment. Your genes can affect how your immune system reacts to a trigger.

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What raises your risk of sarcoidosis?

There are many risk factors for sarcoidosis. Some risk factors, such as where you work, can be changed. But your age, family history, and many other risk factors cannot be changed.

• Age: You can get sarcoidosis at any age, but the risk goes up as you get older, especially after age 55.

• Environment: Living or working near insecticides, mold, or other substances that may cause inflammation raises your risk. You may be around these substances if you are in health care or the automotive industry or are a farmer or firefighter.

• Family history and genetics: Having a close relative with sarcoidosis raises your risk.

• Medicines: Certain types of HIV medicines and monoclonal antibodiesexternal link used to treat cancer or overactive immune system can raise your risk.

• Race or ethnicity: Your risk is higher if you are of African or Scandinavian descent.

• Sex: Women are more likely to have sarcoidosis, although men can also have it.

 

Other medical conditions, such as lymphoma, a type of blood cancer, can also lead to sarcoidosis.

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Treatment

The goal of treatment for sarcoidosis is remission, which means the condition still exists but does not cause problems. Not everyone who is diagnosed with sarcoidosis needs treatment. Sometimes the condition goes away on its own. Your treatment will depend on your symptoms, which organs are affected, and whether those organs are working well.

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Medicine

Your doctor may prescribe medicines to lower inflammation or treat an overactive immune response. Immune-lowering medicines can raise the risk of infections. Talk with your provider about the benefits and risks of these medicines.

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Corticosteroids to treat inflammation

The corticosteroid (steroid hormone medicine) prednisone is the most common treatment for sarcoidosis. Corticosteroids can be taken as pills or be injected, inhaled, or taken as eye drops or other topical medicines.

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Corticosteroids can have serious side effects with long-term use, especially if taken in high doses. Side effects of the corticosteroid pill may include high blood sugar or blood pressure, mood changes, weight gain, and increased appetite. The pill also raises the risk of cataracts (clouding of the eye), glaucoma (damage to a nerve in the eye from high pressure), or osteoporosis (bone thinning). Common side effects from inhaled corticosteroids include a hoarse voice or a mouth infection called thrush.

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Medicines to lower your immune system response

• Medicines used to treat severe rheumatoid arthritis include methotrexate, azathioprine, and leflunomide. You usually take this as a pill or an injection (shot). Side effects may include liver damage or blood problems.

• Monoclonal antibodiesexternal link, also called immunotherapy, are used to treat cancer or an overactive immune system. These include rituximab, infliximab, golimumab, and adalimumab. Your doctor will give you the medicine as a shot or through an IV. Side effects are rare but can include a life-threatening immune reaction, heart problems, low blood counts, or a higher risk of certain cancers.

• Medicines used to treat malaria include hydroxychloroquine or chloroquine. Side effects may include eye damage, heart problems, and low blood sugar.

• Corticotropin (a hormone medicine) is given as a shot. It may be used when prednisone does not work or has serious side effects. Side effects of this medicine may include high blood pressure, problems controlling blood sugar, increased appetite, or mood changes.

• Pentoxifylline is taken as a pill. It is normally prescribed to improve blood flow. Side effects may include nausea.

 

Medicines to treat other symptoms

• Antibiotics treat sarcoidosis of the skin. Examples include minocycline, tetracycline, and doxycycline. Side effects may include dizziness and gastrointestinal tract problems.

• Colchicine treats joint pain from sarcoidosis. You take this medicine as a pill. It is usually prescribed for gout. Side effects include nausea, vomiting, diarrhea, and stomach cramps or pain.

 

Treating complications

If untreated, or if the treatment does not work, sarcoidosis can cause serious health problems. Your doctor may recommend the following medicines or procedures. Ask your doctor about the benefits and risks of any treatment.

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Lung problems

• Inhaled corticosteroids or oxygen therapy help with breathing problems.

• Vasodilator treatment relaxes your blood vessels if you have pulmonary hypertension.

• Pulmonary rehabilitation helps you breathe easier and be more active.

 

Heart problems

• Implantable cardioverter defibrillator (ICD) helps prevent cardiac arrest.

• High blood pressure medicines, such as ACE inhibitors and beta blockers, lowers blood pressure.

 

Hormone problems

• Lifestyle changes, such as avoiding too much sunlight, drinking plenty of fluids, and eating fewer foods with calcium, may be needed if you have too much calcium in the blood or urine.

• Hormone replacement treatment is used to treat some types of sarcoidosis.

 

Brain, nerve, or muscle problems

• Anti-seizure medicines are used if sarcoidosis affects your brain and causes seizures.

• Medicines can be given for nerve or muscle pain.

• Physical therapy improves muscle strength.

• Surgery removes brain tumors.

 

Severe organ damage

• Transplant surgery can be performed if sarcoidosis causes life-threatening lung, heart, or liver damage.

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Living with Sarcoidosis

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Manage your condition

Even if you do not have symptoms of sarcoidosis, you should see your healthcare provider for ongoing care. For example, your provider will monitor you for side effects from long-term use of corticosteroids or other medicines.

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If the disease is not worsening, you may be watched closely to see whether the disease goes away on its own. If the disease does start to get worse, your doctor can prescribe treatment.

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Remission and flares

If your sarcoidosis goes into remission, your doctor may carefully stop your medicines. However, you will still need to watch for a flare. If you do have a flare, you may need another round of treatment.

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Flares can be hard to predict. Most often, they happen within six months of stopping treatment. The longer you go without symptoms, the less likely you are to have a flare.

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Tests for complications

Some people have sarcoidosis that persists or comes back for many years after diagnosis. This may be called chronic, severe, advanced, refractory, or progressive sarcoidosis. Your provider may order tests to keep track of your condition and check for complications. .

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Know when to seek medical care

Watch for the warning signs of complications that may require emergency medical treatment. These include signs of changes in vision that may be a sign of brain tumors. Other complications that require immediate medical attention include kidney failure, cardiac arrest, and sudden shortness of breath or muscle weakness.

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Make healthy lifestyle changes

A healthy lifestyle may help you feel better and prevent sarcoidosis from getting worse.

• Get regular physical activity. Extreme tiredness can make it hard to exercise if you have sarcoidosis. However, physical activity can improve energy and help with other symptoms, such as shortness of breath and muscle weakness. Try to stay active but talk with your doctor about what level of physical activity is right for you.

• Quit smoking. If you smoke, quit. Also, try to avoid other lung irritants, such as dust, chemicals, and secondhand smoke. Visit Smoking and Your Heart and our Your Guide to a Healthy Heart. For free help quitting smoking, you may call the National Cancer Institute's Smoking Quitline at 1-877-44U-QUIT (1-877-448-7848).

• Try to get enough good quality sleep. Experts recommend that adults get 7 to 9 hours of sleep per day.

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Take care of your mental health

Sarcoidosis may make you feel lonely, anxious, or depressed. You may continue to feel very tired even after your treatment has ended. But certain activities or treatments may help improve your mental health.

• Counseling, particularly cognitive therapy, can be helpful.

• Joining a patient support group may help you adjust to living with sarcoidosis. You can see how other people manage similar symptoms and their condition. Talk with your provider about local support groups or check with an area medical center.

• Talk to your doctor about medicines or other treatments. Antidepressants or other treatments may improve your quality of life.

• Support from family and friends can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you.

 

Pregnancy and Sarcoidosis

Most women who have sarcoidosis give birth to healthy babies. However, they have a higher risk of certain complications, including:

• Heavy bleeding after giving birth

• Preterm delivery

• Preeclampsia, which is high blood pressure and too much protein in the urine

• Venous thromboembolism, which is a blood clot in the lungs or deep veins, usually in the legs

If you have sarcoidosis and are pregnant or planning to become pregnant, talk with your doctor. It is important to get good prenatal care and regular sarcoidosis checkups before, during, and after pregnancy. Also, tell your doctor about any medicines you take. Some sarcoidosis medicines are not safe to take during pregnancy.

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Bronchiectasis

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What is bronchiectasis?

Bronchiectasis is a condition that occurs when the tubes that carry air in and out of your lungs get damaged, causing them to widen and become loose and scarred. These tubes are called airways.

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Bronchiectasis usually results from an infection or other condition that injures the walls of your airways or prevents the airways themselves from clearing mucus. Mucus is a slimy substance that the airways produce to help remove inhaled dust, bacteria, and other small particles.

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In bronchiectasis, your airways slowly lose their ability to clear out this mucus. When mucus can't be cleared, it builds up and creates an environment in which bacteria can grow. This leads to repeated, serious lung infections.

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Each infection causes more damage to your airways. Over time, the airways lose their ability to move air in and out. This can make it hard for enough oxygen to reach your vital organs.

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Bronchiectasis can affect just one section of one of your lungs or many sections of both lungs. It can lead to serious health problems, such as respiratory failure, a collapsed lung, and heart failure. Currently, bronchiectasis has no cure. However, with proper care, most people who have it can enjoy a good quality of life.

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Early diagnosis and treatment of bronchiectasis is important. The sooner your doctor starts treating bronchiectasis and any underlying conditions, the better your chances of preventing further lung damage.

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Symptoms

The initial airway damage that leads to bronchiectasis often begins in childhood. However, symptoms may not appear until months or even years after you start having repeated lung infections.

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The most common symptoms of bronchiectasis are:

• A daily cough that occurs over months or years

• Daily production of large amounts of sputum (spit), which you cough up and which may have mucus, trapped particles, and pus

• Shortness of breath and wheezing (a whistling sound when you breathe)

• Chest pain

• Clubbing (the flesh under your fingernails and toenails gets thicker, causing the nails to curve downward)

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If your doctor listens to your lungs with a stethoscope, he or she may hear abnormal lung sounds.

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Over time, you may have more serious symptoms. You may cough up blood or bloody mucus and feel very tired. Children may lose weight or not grow at a normal rate.

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How will my doctor find out if I have bronchiectasis?

Your doctor may think you have bronchiectasis if you have a daily cough that produces large amounts of sputum (spit).

To find out whether you have bronchiectasis, your doctor may recommend tests to:

• Find any underlying causes that require treatment

• Rule out other causes of your symptoms

• Find out how much your airways are damaged

 

Diagnostic tests and procedures

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Chest CT scan

A chest computed tomography (CT) scan is a painless imaging test that takes many detailed pictures, called slices, of your lungs and the inside of your chest. Computers can combine these pictures to create three-dimensional (3D) models that show the size, shape, and position of your lungs and structures in your chest.

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A chest CT scan can help figure out the cause of lung symptoms such as shortness of breath or chest pain. It can also tell your doctor if you have certain lung problems such as a tumor, excess fluid around the lungs that is known as pleural effusion, or pneumonia.  Your chest CT scan may be done in a medical imaging facility or hospital. You will lie still on a table and the table will slide into the scanner. You will hear soft buzzing or clicking sounds when you are inside the scanner and the scanner is taking pictures. You will be able to hear from and talk to the technician performing the test while you are inside the scanner. For some diagnoses, a contrast dye, often iodine-based, may be injected into a vein in your arm before the imaging test. In rare instances, some people have an allergic reaction to contrast dye. There is also a slight risk of cancer, particularly in growing children, because the test uses radiation. Although the amount of radiation from one test is usually less than the amount of radiation you are naturally exposed to over three years, patients should not receive more CT scans than what is recommended by clinical guidelines recommend.

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Chest X-ray

A chest X-ray is a fast and painless imaging test to look at the structures in and around your chest. This test can help diagnose and check conditions such as pneumonia, heart failure, lung cancer, tuberculosis, sarcoidosis, and lung tissue scarring, called fibrosis. Doctors may use chest X-rays to see how well certain treatments are working and to check for complications after certain procedures or surgeries. The test may be done in the doctor’s office, clinic, or hospital. You will stand, sit, or lie still for the test. Chest X-rays have few risks. The amount of radiation used in a chest X-ray is very small. Talk to your provider if you are or could be pregnant.

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Other tests

Your doctor may recommend other tests.

• Blood tests can show whether you have an underlying condition that can lead to bronchiectasis. Blood tests also can show whether you have an infection or low levels of certain infection-fighting blood cells.

• A sputum culture shows whether a sample of your sputum has bacteria (such as the bacteria that cause tuberculosis) or fungi.

• Lung function tests measure how much air you can breathe in and out, how fast you can breathe air out, and how well your lungs deliver oxygen to your blood. Lung function tests help show how much lung damage you have.

• A sweat test or other tests can help determine if you have cystic fibrosis.

 

Bronchoscopy

If your bronchiectasis doesn't respond to treatment, your doctor may recommend bronchoscopy. Doctors use this procedure to look inside the airways.

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During bronchoscopy, a flexible tube with a light on the end is inserted through your nose or mouth into your airways. The tube is called a bronchoscope. It provides a video image of your airways. You'll be given medicine to numb your upper airway and help you relax during the procedure.

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Bronchoscopy can show whether you have a blockage in your airways. The procedure also can show the source of any bleeding in your airways.

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Causes and Risk Factors

In the United States, childhood infections such as whooping cough and measles used to cause many cases of bronchiectasis. However, these infections are now less common because of vaccines and antibiotics.

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These days, bronchiectasis usually is caused by medical conditions such as cystic fibrosis and primary ciliary dyskinesia, that injure the airway walls or prevent the airways from clearing mucus.

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Bronchiectasis can develop at any age. Overall, two-thirds of people who have the condition are women. However, in children, the condition is more common in boys than in girls.

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How do you get it?

Bronchiectasis can be congenital or acquired.

• Congenital bronchiectasis affects infants and children. It's the result of a problem with how the lungs form in a fetus.

• Acquired bronchiectasis occurs as a result of another condition or factor. This type of bronchiectasis can affect adults and older children. Acquired bronchiectasis is more common than the congenital type.

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Damage to the walls of the airways – for example, from a lung infection – usually causes acquired bronchiectasis. Examples of lung infections that can lead to bronchiectasis include:

• Severe pneumonia  

• Whooping cough or measles (uncommon in the United States due to vaccination)

• Tuberculosis

• Fungal infections

 

Other conditions, such as an airway blockage, also can lead to bronchiectasis. Many things can cause a blockage, such as a growth or a noncancerous tumor. An inhaled object, such as a piece of a toy or a peanut that you inhaled as a child, also can cause an airway blockage.

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What raises your risk?

People who have conditions that damage the lungs or increase the risk of lung infections are at risk of bronchiectasis. Examples of such conditions include:

• Cystic fibrosis, which causes almost half of the cases of bronchiectasis in the United States

• Immunodeficiency disorders, such as common variable immunodeficiency and, less often, HIV and AIDS

• Allergic bronchopulmonary aspergillosis, which is an allergic reaction to a fungus called aspergillus that causes swelling in the airways

• Disorders that affect cilia function, such as primary ciliary dyskinesia, can cause bronchiectasis. Cilia are small, hair-like structures that line your airways. They help clear mucus (a slimy substance) out of your airways.

• Chronic (long-term) pulmonary aspiration, which can inflame the airways

• Connective tissue diseases, such as rheumatoid arthritis, Sjögren’s syndrome, and Crohn’s disease

 

Can you prevent bronchiectasis?

To prevent bronchiectasis, it's important to prevent the lung infections and lung damage that can cause it.

• Get childhood vaccines for measles and whooping cough. These vaccines also reduce complications from these infections, such as bronchiectasis.

• Avoid toxic fumes, gases, smoke, and other harmful substances to help protect your lungs.

• Treat lung infections in children to help preserve lung function and prevent lung damage that can lead to bronchiectasis.

• Stay alert to keep children (and adults) from inhaling small objects (such as pieces of toys and food that might stick in a small airway). If you think you, your child, or someone else has inhaled a small object, seek medical care right away.

 

In some cases, treating the basic cause of bronchiectasis can slow or prevent its progression.

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Treatment

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How is bronchiectasis treated?

Bronchiectasis often is treated with medicines, hydration, and chest physical therapy (CPT). Your doctor may recommend surgery if the bronchiectasis is isolated to a section of lung, or you have a lot of bleeding.

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If the bronchiectasis is widespread and causing respiratory failure, your doctor may recommend oxygen therapy.

The goals of treatment are to:

• Treat any underlying conditions and lung infections

• Remove mucus (a slimy substance) from your lungs

• Prevent complications

 

Early diagnosis and treatment of the underlying cause of bronchiectasis may help prevent further lung damage. 

 

Medicines

Antibiotics

Antibiotics are the main treatment for the repeated lung infections that bronchiectasis causes. Oral antibiotics often are used to treat these infections.

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For hard-to-treat infections, your doctor may prescribe intravenous (IV) antibiotics. These medicines are given through an IV line inserted into your arm. Your doctor may help you arrange for a home care provider to give you IV antibiotics at home.

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Expectorants and mucus-thinning medicines

Your doctor may prescribe expectorants and mucus thinners to help you cough up mucus.

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Expectorants help loosen the mucus in your lungs. They often are combined with decongestants, which may provide extra relief.

 

Mucus thinners, such as acetylcysteine, loosen the mucus to make it easier to cough up.

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Other medicines

Your doctor may prescribe other medicines, depending on your symptoms or other conditions you may have.

• Bronchodilators relax the muscles around your airways. This helps open your airways and makes breathing easier. Most bronchodilators are inhaled medicines. You will use an inhaler or a nebulizer to breathe in a fine mist of medicine. Inhaled bronchodilators work quickly because the medicine goes straight to your lungs. Your doctor may recommend that you use a bronchodilator right before you do CPT.

• Inhaled corticosteroids treat inflammation in the airways. Your doctor may prescribe these if you also have wheezing or asthma with your bronchiectasis.

 

Hydration

Drinking plenty of fluid, especially water, helps prevent airway mucus from becoming thick and sticky. Good hydration helps keep airway mucus moist and slippery and easier to cough up.

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Chest physical therapy

CPT is also known as physiotherapy, chest clapping, and percussion. This technique is generally performed by a respiratory therapist but can be done by a trained member of the family. It involves the therapist patting or gently pounding your chest and back repeatedly with their hands or a device. Doing this helps loosen the mucus from your lungs so you can cough it up.

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You can sit with your head tilted down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs.

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Some people who perform CPT find it hard or uncomfortable to do. Several devices can help with CPT, such as:

• An electric chest clapper, known as a mechanical percussor

• An inflatable therapy vest that uses high-frequency air waves to force mucus toward your upper airways so you can cough it up

• A small handheld device that you breathe out through, which causes vibrations that dislodge the mucus

• A mask that creates vibrations to help break loose mucus from your airway walls

 

Several breathing techniques are also used to help move mucus to the upper airway so it can be coughed up. These techniques include forced expiration technique (FET) and active cycle breathing (ACB).

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FET involves forcing out a couple of breaths and then doing relaxed breathing. ACB is FET that involves deep breathing exercises.

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Oxygen therapy

Oxygen therapy is a treatment that delivers oxygen for you to breathe. You can receive oxygen therapy from tubes resting in your nose, a face mask, or a tube placed in your trachea (windpipe). You may need oxygen therapy if you have a condition that causes your blood oxygen levels to be too low. 

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Oxygen therapy can be given for a short or long period of time in the hospital, in another medical setting, or at home. Oxygen poses a fire risk, so you should never smoke or use flammable materials when using oxygen. You may experience side effects from this treatment, such as a dry or bloody nose, tiredness, and morning headaches. Oxygen therapy is generally safe. 

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Surgery

Your doctor may recommend surgery if no other treatments have helped and only one part of your airway is affected. If you have major bleeding in your airway, your doctor may recommend surgery to remove part of your airway or a procedure to control the bleeding.

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In very rare instances of severe bronchiectasis, your doctor may recommend that you receive a lung transplant replacing your diseased lungs with a healthy set of lungs.

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Living with bronchiectasis

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Early diagnosis and treatment of bronchiectasis can prevent further damage to your lungs. People who have bronchiectasis should get ongoing medical care and try to follow a healthy lifestyle.

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Ongoing care

If you have bronchiectasis, work closely with your doctor to learn how to improve your quality of life. This involves learning as much as you can about bronchiectasis and any underlying conditions that you have.

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Take steps to avoid lung infections. Ask your doctor about getting flu and pneumonia vaccines. Wash your hands often to lower your risk of getting viruses and bacterial infections.

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Healthy lifestyle

Following a healthy lifestyle is important for overall health and well-being.

• Quit smoking. Talk with your doctor about programs and products that can help you quit smoking. Also, try to avoid secondhand smoke. If you have trouble quitting smoking on your own, consider joining a support group. Many hospitals, workplaces, and community groups offer classes to help people quit smoking.

• Avoid toxic fumes, gases, and other harmful substances.

• Choose healthy foods. A healthy diet includes a variety of vegetables and fruits. It also includes whole grains, fat-free or low-fat dairy products, and protein foods, such as lean meats, poultry without skin, seafood, processed soy products, nuts, seeds, beans, and peas.

• Stay hydrated. Drinking plenty of fluids, especially water, helps prevent airway mucus from becoming thick and sticky.

• Be physically active. Physical activity, such as walking and swimming, can help loosen mucus. Ask your doctor what types and amounts of activity are safe for you.

 

Take care of your mental health

People who have chronic lung diseases are more prone to depression, anxiety, and other emotional problems. Talk about how you feel with your healthcare team. Talking to a professional counselor can also help. If you’re very depressed, your doctor may recommend medicines or other treatments that can improve your quality of life.

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Joining a patient support group may help you adjust to living with bronchiectasis. You can see how other people who have the same symptoms have coped with them. Talk with your doctor about local support groups or check with an area medical center.

Support from family and friends also can help relieve stress and anxiety. Let your loved ones know how you feel and what they can do to help you. 

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Serious health problems from bronchiectasis

Severe bronchiectasis can lead to other serious health conditions.

• Respiratory failure develops when not enough oxygen passes from your lungs into your blood.

• Atelectasis occurs when one or more areas of your lungs collapse or don't inflate properly. As a result, you may feel short of breath. Your heart rate and breathing rate may increase, and your skin and lips may turn blue.

• Heart failure can occur if bronchiectasis is so advanced that it affects all parts of your airways. Heart failure develops when the heart can't pump enough blood to meet the body's needs. The most common symptoms of heart failure are shortness of breath or trouble breathing; tiredness; and swelling in the ankles, feet, legs, abdomen, and veins in the neck. swelling in the ankles, feet, legs, abdomen, and veins in the neck.

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Pleural Disorders

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Pleural disorders are conditions that affect the tissue that covers the outside of the lungs and lines the inside of your chest cavity.

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The tissue is called the pleura, and the thin space between its two layers is called the pleural space. A small amount of fluid fills the pleural space, and when you breathe in and out, this fluid helps the pleural layers glide smoothly against each other. An injury, inflammation, or infection can cause the blood or air to build up in the pleural space and lead to a pleural disorder.

There are three types of pleural disorders — pleurisy, pleural effusion, and pneumothorax — and they have varying causes. Pleurisy is inflammation of the pleura. Pleural effusion and pneumothorax occur when an infection, medical condition, or chest injury causes fluid, pus, blood, air, or other gases to build up in the pleural space.

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Chest pain, shortness of breath, and coughing are common symptoms of all types of pleural disorders, but treatment for pleural disorders varies depending on what type you have and how serious it is. If left untreated, pleural disorders can lead to serious problems, including complete collapse of the lung, shock, or sepsis.

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The three types of pleural disorders include pleural effusion, pleurisy, and pneumothorax.

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What is pleural effusion?

Pleural effusion is a buildup of fluid in the pleural space. The cause of the buildup determines the type of pleural effusion.

• Exudative effusion is caused by a buildup of fluid from inflammation, tumors, infection, or lung injury. The types of exudative effusion vary by the fluid buildup in the pleural space. For example, an empyema is a buildup of infection or pus, a hemothorax happens when blood builds up, and a chylothorax results from a buildup of chyle, a substance formed in the small intestine.

• Transudative pleural effusion is caused by pressure in the blood vessels, most often because of a medical condition such as heart, kidney, or liver failure. The pressure pushes excess fluid into the pleural cavity.

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What is pleurisy?

Pleurisy is inflammation of the pleura. It is also called pleuritis.

 

What is pneumothorax?

A pneumothorax is a collection of air or gas in the pleural space. The buildup of air or gas can put pressure on the lung and cause all or part of it to collapse.

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There are different types of pneumothorax.

• Spontaneous pneumothorax can happen suddenly and without any known cause or in people without any signs of lung disease. Sometimes, it may be caused by another medical condition that affects the lungs, such as chronic obstructive pulmonary disease (COPD).

• Tension pneumothorax is a very large pneumothorax that may result in failure of the heart and the lungs. This can interfere with blood flow through your chest and cause your blood pressure to drop.

• Traumatic pneumothorax is caused by a chest injury.

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Symptoms

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Pleural disorders may occur with no symptoms, or they may have obvious symptoms. Symptoms and complications vary depending on the pleural disorder and how severe it is.

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Symptoms for pleural disorders may include:

• Chest pain that is sharp or stabbing and gets worse when you breathe in deeply or cough or sneeze

• Cough

• Shortness of breath

• Low weight or slow rate of weight gain in children: Usually the weight is well below the child’s ideal weight or the average weight of other children of the same age.

• Fever

• Fatigue

• Bluish skin caused by not getting enough oxygen

• Anxiety

• Rapid heart rate

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Diagnosis

Your healthcare provider will want to learn about your symptoms and risk factors and do diagnostic tests or procedures to determine whether you have a pleural disorder and, if so, what type.

 

Medical history

To help diagnose a pleural disorder, your healthcare provider may ask you about your personal and family health history. They will also ask you to describe any chest pain, including details such as the following:

• What it feels like

• Where it is located and whether you can feel it in your arms, jaw, or shoulders

• When it started and whether it goes away and then comes back

• What makes it better or worse

 

This information about the chest pain you have experienced can help your provider determine whether it is caused by problems with your heart, chest muscles, lungs, or digestive system.

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Physical exam

As part of a physical examination, your provider will measure your blood pressure and heart rate, feel your chest and belly, take your temperature, listen to your heart and lungs, and feel your pulse. They may also check the level of oxygen in your blood with a probe on your finger or forehead.

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Your provider will listen to your breathing to find out whether your lungs are making any abnormal sounds.

• If you have pleurisy, the inflamed layers of the pleura may make a rough, scratchy sound as they rub against each other when you breathe. Doctors call this a pleural friction rub.

• If you have a pleural effusion, fluid buildup in the pleural space may prevent a friction rub. But if you have a lot of fluid, your provider may hear a dull sound when they tap on your chest.

• If you have a pneumothorax, your provider may hear more echo than usual when they tap on your chest.

 

Diagnostic tests and procedures

Your provider may order a combination of the following tests to help diagnose a pleural disorder. These tests may also help  rule out other medical conditions that can cause chest pain.

• Biopsies take a sample of the pleura. The sample is checked for signs of disease.

• Blood tests show whether you have an illness that increases your risk of pleurisy or another pleural disorder

• Lung imaging tests such as a chest MRI or chest X-ray look for fluid or air in the pleural space or other possible causes of pleural disorders, such as pneumonia, a fractured rib, or a lung tumor.

• Endoscopies look for signs of disease, guide the doctor while performing a biopsy, or remove pleural fluid.

• Thoracentesis procedure removes a sample of pleural fluid for testing. The fluid removed during thoracentesis is tested and examined under a microscope for signs of infection, cancer, or other conditions that can cause fluid or blood to build up in the pleural space.

• Ultrasounds look for fluid, air, or other abnormal findings in your chest.

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Causes and Risk Factors

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Pleural disorders may be caused by inflammation, injury, or an imbalance of fluids in the pleural space. Your age and family history may increase your risk. You may also be at higher risk if you take certain medicines, smoke tobacco or marijuana, or have other medical conditions. You may be able to help prevent pleural disorders by quitting smoking or not starting if you don’t smoke.

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What causes pleural disorders?

Pleural disorders may be caused by one of the following.

• Inflammation in the pleura: The inflammation may happen because of an infection, tumor, or another medical condition. The inflammation affects the two thin layers of the pleura. This can cause the surface of the layers to become rough and the fluid in between the layers to become sticky. When this happens, the two layers may rub together every time you breathe in instead of gliding past each other.

• Injury to the chest: Even an injury that does not break the skin but causes internal damage, can allow air, fluid, or blood to leak into the pleural space. This can cause a pneumothorax or pleural effusion.

• Procedure such as chest biopsy, mechanical ventilation, or thoracentesis: This can cause blood or air to leak into the pleural space.

• Fluid imbalance: Pleural effusions develop when fluid enters the pleural space faster or at a higher amount than the body can reabsorb. This imbalance of fluids can be caused by heart, kidney, or liver failure, or other medical conditions.

 

Who is at risk?

Your risk for pleural disorders may be higher based on some factors you can control and some, like your age, that you can't control. 

• Age: Pleural disorders can happen at any age, but pneumothorax is most common in people 15 to 34 years old and people over age 55.

• Family history: If someone in your family had spontaneous pneumothorax, your risk of having this disease is higher.

• Lifestyle habits: Smoking tobacco or marijuana raise your risk of spontaneous pneumothorax.

• Medical history: The medicines you take may raise your risk, including blood thinners, cancer medicines, and nitrofurantoin, an antibiotic to treat urinary tract infections. Some health conditions may also raise the risk for pleural disorders, such as cancer, heart failure, autoimmune diseases, kidney disease, lung disease, and sickle cell disease.

• Sex: Men, especially those who are taller than average, are more likely than women to have a spontaneous pneumothorax, often without underlying lung disease.

 

Can you prevent pleural disorders?

Because your lifestyle habits and other medical conditions can raise your risk of pleural disorders, your doctor may talk to you about steps you can take to prevent them. 

• Treating conditions that increase the risk of pleural disorders

• Quitting smoking

• Quitting or avoiding illegal drugs, such as marijuana

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Treatment

 

Call 9-1-1 if you have severe chest pain or trouble breathing. These are signs of a tension pneumothorax, which can lead to shock, a life-threatening medical emergency. 

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How your pleural disorder is treated depends on what type of pleural disorder you have and how severe it is. Some pleural disorders go away without treatment. Others will require a procedure to remove air, fluid, or other material from the pleural space. The goal of treatment is to relieve symptoms and treat the underlying condition.

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Medicines

Your healthcare provider may recommend medicine to treat symptoms or causes of your pleural disorder.

• Antibiotics, antifungals, or antiparasitic medicines treat infections in the pleural space or in the lung.

• Corticosteroids reduce inflammation. Corticosteroids can have serious side effects with long-term use.

• Morphine in low doses is used to treat chronic (long-term) shortness of breath. This medicine has a risk of addiction.

• Nonsteroidal anti-inflammatory drugs such as ibuprofen help reduce pain and inflammation.

 

Procedures

Your doctor may perform one or more procedures to treat a pleural disorder. Many of these procedures are performed using ultrasound.

• Chest tubes drain fluid, blood, or air from the pleural space. This process can take several days. You may stay in the hospital while the tube is in place.

• Heimlich valves prevent fluid and air from getting into the chest when you breathe in. Your doctor may attach this one-way valve to a chest tube or indwelling pleural catheter (IPC), which allows you to move around more and may allow you to go home to wait for your lung to re-expand.

• IPC, or indwelling pleural catheters, are used to drain pleural fluid. An IPC is a semi-permanent thin tube that is left in place so patients or caregivers can drain the fluid, usually a few times a week.

• Injection of medicines into the pleural space may be needed to break up material that cannot be removed with a needle or drained through a chest tube.

• One-way endobronchial valve may be implanted in one of your bronchial tubes to allow air to exit the pleural space but not reenter.

• Pleurodesis is a procedure that can help close the pleural space by helping the two sides of the pleura stick together. For this procedure, your doctor will drain the fluid out of your chest through a chest tube. Then they will push a substance through the chest tube into the pleural space. The substance will irritate the surface of the pleura and cause the two layers of the pleura to stick together, preventing more fluid from building up.

• Surgery may be needed to remove fluid, pus, or blood clots that cannot be removed with a chest tube. You may also need surgery to remove part of the pleura, to remove one or more ribs; or to close the pleural space with a pleurodesis procedure.

• Thoracentesis is a procedure to remove air, blood, or other fluid from the pleural space with a needle. Possible complications include pneumothorax, pain, hemothorax and other bleeding, infection, and pulmonary edema. Complications are less likely if ultrasound is used to guide the procedure.

 

Oxygen Therapy

You may need oxygen therapy for a pneumothorax. Oxygen therapy is a treatment that delivers oxygen gas for you to breathe. You can receive oxygen therapy from tubes resting in your nose, a face mask, or a tube placed in your trachea, or windpipe. This treatment increases the amount of oxygen your lungs receive and deliver to your blood.

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What happens if pleural disorders are left untreated?

If left untreated, or if they do not respond well to treatment, pleural disorders can lead to serious health problems. Some of the possible complications include:

• Atelectasis, a partial or complete collapse of the lung, which can be a complication of pneumothorax

• Empyema, which is pus in the pleural space

• sepsis

• Shock, which is a life-threatening complication of hemothorax or tension pneumothorax that can happen when blood pressure drops dangerously low

• Unexpandable (trapped or entrapped) lung, which can be a complication of pleural effusion that is caused by cancer, hemothorax, or pleurisy

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Recovery

As you recover from your treatment for a pleural disorder, it is important to follow your treatment plan. You may need to follow up with your healthcare provider regularly to monitor your condition. You will also want to take steps to prevent complications or a repeat pleural disorder.

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Receive routine follow-up care

If your pleural disorder is caused by a disease such as COPD, be sure to follow the treatment plan for that condition.

• Talk to your provider about how often you need to schedule follow-up visits. To monitor your condition, your doctor may recommend a chest X-ray 6 weeks after treatment.

• See your provider a few weeks after a pneumothorax to make sure it has cleared up.

• If you have a Heimlich valve, ask your provider how often it needs to be inspected by them.

 

If you have an IPC, or indwelling pleural catheter, to drain pleural fluid, it may stay in for a few months or longer. Infection is possible around the insertion site, but infections can usually be treated without removing the IPC.

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Learn about other precautions to help you stay safe

If you have or have recently had a pneumothorax, your provider may recommend the following precautions to help you stay safe.

• Avoid strenuous exercise and contact sports until your provider says it is safe.

• Do not fly. Ask your provider when it is safe again to fly.

• Do not go scuba diving unless your provider says you have zero risk of recurrence. If a pneumothorax recurs during diving, it can cause a tension pneumothorax, which is a life-threatening complication.

• Tell your doctor if you are pregnant. Talk to your doctor about how to manage your delivery so that the pneumothorax does not get worse. Also talk about whether you should have surgery later to prevent another pneumothorax in a future pregnancy.

 

Prevent a repeat spontaneous pneumothorax event

After a spontaneous pneumothorax, you are at risk of having another spontaneous pneumothorax. People who have underlying lung disease are more likely to have another spontaneous pneumothorax.

Quitting smoking if you smoke and not starting if you do not smoke can help reduce your risk.

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