Pulmonary Disease & Critical Care
Associates, PA
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Acute Respiratory Syndrome (ARDS) Alpha-1-Antitrypsin Deficiency Bronchopulmonary Dysplasia (BPD) Chronic Obstructive Pulmonary Disease (See COPD) Hemothorax (See Pleurisy and other disorders of the pleura) What is LAM (Lymphangioleiomyomatosis) Pleurisy and Other Disorders of the Pleura Pulmonary Arterial Hypertension
ARDS, or acute respiratory distress syndrome, is a lung condition that leads to low oxygen levels in the blood. ARDS can be life threatening. This is because your body's organs, such as the kidneys and brain, need oxygen-rich blood to work properly. ARDS usually occurs in people who are very ill with another disease or who have major injuries. Most people are already in the hospital when they develop ARDS. What Is Alpha-1 Antitrypsin Deficiency?Alpha-1 antitrypsin (an-tee-TRIP-sin) deficiency, or AAT deficiency, is a condition that raises your risk for certain types of lung disease, especially if you smoke. AAT deficiency is an inherited condition. "Inherited" means it's passed in the genes from parents to children. Some people who have severe AAT deficiency develop emphysema (em-fi-SE-ma)—often when they're only in their forties or fifties. Emphysema is a serious lung disease in which damage to the airways makes it hard to breathe. A smaller number of people who have AAT deficiency have cirrhosis (sir-RO-sis) and other serious liver diseases. Cirrhosis is a disease in which the liver is scarred. This prevents the liver from working properly. In people who have AAT deficiency, cirrhosis and other liver diseases usually occur in infancy and early childhood. A very small number of people who have AAT deficiency have a rare type of skin disease called necrotizing panniculitis (pa-NIK-yu-LI-tis). This skin disease can cause painful lumps under or on the surface of the skin What Is Asthma?Asthma (Az-muh) is a chronic disease that affects your airways. The airways are the tubes that carry air in and out of your lungs. If you have asthma, the inside walls of your airways are inflamed (swollen). The inflammation (IN-fla-MAY-shun) makes the airways very sensitive, and they tend to react strongly to things that you are allergic to or find irritating. When the airways react, they get narrower, and less air flows through to your lung tissue. This causes symptoms like wheezing (a whistling sound when you breathe), coughing, chest tightness, and trouble breathing, especially at night and in the early morning. Asthma cannot be cured, but most people with asthma can control it so that they have few and infrequent symptoms and can live active lives. When your asthma symptoms become worse than usual, it is called an asthma episode or attack. During an asthma attack, muscles around the airways tighten up, making the airways narrower so less air flows through. Inflammation increases, and the airways become more swollen and even narrower. Cells in the airways may also make more mucus than usual. This extra mucus also narrows the airways. These changes make it harder to breathe What Is Bronchiectasis?Bronchiectasis (bron-kee-ek'-tas-is) is a lung disease that usually results from an infection or other condition that injures the walls of the airways in your lungs. The airways are the tubes that carry air in and out of your lungs. This injury is the beginning of a cycle in which your airways slowly lose their ability to clear out mucus. The mucus builds up and creates an environment in which bacteria can grow. This leads to repeated serious lung infections. Each infection causes more damage to your airways. Over time, your airways become stretched out, flabby, and scarred. They can no longer move air in and out. This can affect how much oxygen reaches your body organs. If your lungs cannot move enough oxygen into your body, bronchiectasis can lead to serious illness, including heart failure. Bronchiectasis can affect just one section of one of your lungs or many sections of both lungs. Bronchiectasis usually begins in childhood, but symptoms may not appear until months or even years after you have started having repeated lung infections. There are two types of bronchiectasis:
Bronchiectasis cannot be cured, but with proper care, most people who have it can enjoy a good quality of life What Is Bronchopulmonary Dysplasia?Bronchopulmonary dysplasia, or BPD, is a serious lung condition that affects mostly babies who:
Many of these babies are born with serious respiratory distress syndrome (RDS). Their lungs haven't yet developed enough to make surfactant (sur-FAK-tant). Surfactant is a liquid that coats the inside of the lungs and keeps them open so that the baby can breathe in air once he or she is born. As a result, these babies are usually put on oxygen and a breathing machine at birth—either a ventilator (also known as a respirator) or a nasal continuous positive airway pressure (NCPAP) machine. This can prevent damage to their brains and other body organs from lack of oxygen. They also are given surfactant. Most babies with RDS begin to get better within the next 2 to 4 weeks. But some get worse and need more oxygen and/or breathing assistance from a machine. These babies have developed BPD. The lungs of the babies who are born with RDS and go on to develop BPD are less developed than those of babies with RDS who recover. They usually have fewer and larger alveoli, or air sacs, than other newborns. They also may have fewer tiny blood vessels in the alveoli. The blood vessels are needed to move oxygen from the alveoli into the bloodstream. These babies also are more likely than other infants to have problems in other parts of their bodies that aren't yet fully developed. These include the heart, kidneys, brain, stomach, intestines, and eyes. With new and better treatments now available, most babies with BPD get better over time, and many go on to live normal, active lives. Bronchoscopy (bron-KOS-ko-pee) is a procedure used to look inside the lungs' airways, called the bronchi and bronchioles. The airways carry air from the trachea (TRA-ke-ah), or windpipe, to the lungs. During the procedure, your doctor passes a thin, flexible tube called a bronchoscope through your nose (or sometimes your mouth), down your throat, and into the airways. If you have a breathing tube, the bronchoscope can be passed through it to your airways. At the bronchoscope's tip are a light and a mini-camera, so the doctor can see your windpipe and airways. You will be given medicine to make you relaxed and sleepy during the procedure. If there's a lot of bleeding in your lungs or a large object stuck in your throat, your doctor may use a bronchoscope with a rigid tube. The rigid tube, which is passed through the mouth, is wider. This allows your doctor to see inside it more easily, treat bleeding, and remove stuck objects. A rigid bronchoscopy is usually done in a hospital operating room while you're under general anesthesia. Anesthesia is used so you will sleep through the procedure and not feel any pain. What Is a Chest CT Scan?A chest computed tomography (to-MOG-ra-fee), or CT, scan is a painless, noninvasive test. It creates precise images of the structures in your chest, such as your lungs. “Noninvasive” means that no surgery is done and no instruments are inserted into your body. A chest CT scan is a type of x ray. However, a CT scan’s pictures show more details than pictures from a standard chest x ray. Like other x-ray tests, chest CT scans use a form of energy called ionizing radiation. This energy helps create pictures of the inside of your chest. What Is Chest MRI?Chest magnetic resonance imaging (MRI) is a safe, noninvasive test. “Noninvasive” means that no surgery is done and no instruments are inserted into your body. This test creates detailed pictures of the structures in your chest, like your chest wall, heart, and blood vessels. Chest MRI uses radio waves, magnets, and a computer to create these pictures. The test is used to:
As part of some chest MRIs, a special substance (called contrast dye) is injected into a vein in your arm. This dye allows the MRI to take more detailed pictures of the structures in your chest. Chest MRI has few risks. Unlike a CT scan or standard x ray, MRI doesn’t use radiation or have any risk of causing cancer. Rarely, the contrast dye used for some chest MRIs may cause an allergic reaction. What Is Chest X Ray?A chest x ray is a painless, noninvasive test that creates pictures of the structures inside your chest, such as your heart and lungs. "Noninvasive" means that no surgery is done and no instruments are inserted into your body. This test is done to find the cause of symptoms such as shortness of breath, chest pain, chronic cough (a cough that lasts a long time), and fever. What Is COPD?Chronic obstructive pulmonary disease (COPD) is a lung disease in which the lungs are damaged, making it hard to breathe. In COPD, the airways—the tubes that carry air in and out of your lungs—are partly obstructed, making it difficult to get air in and out. Cigarette smoking is the most common cause of COPD. Most people with COPD are smokers or former smokers. Breathing in other kinds of lung irritants, like pollution, dust, or chemicals, over a long period of time may also cause or contribute to COPD. The airways branch out like an upside-down tree, and at the end of each branch are many small, balloon-like air sacs called alveoli (al-VEE-uhl-EYE). In healthy people, each airway is clear and open. The air sacs are small and dainty, and both the airways and air sacs are elastic and springy. When you breathe in, each air sac fills up with air like a small balloon; when you breathe out, the balloon deflates and the air goes out. (See the How the Lungs Work section for details.) In COPD, the airways and air sacs lose their shape and become floppy. Less air gets in and less air goes out because:
COPD
The illustration show the respiratory system and cross-sections of healthy alveoli and alveoli with COPD. COPD develops slowly, and it may be many years before you notice symptoms like feeling short of breath. Most of the time, COPD is diagnosed in middle-aged or older people. COPD is a major cause of death and illness, and it is the fourth leading cause of death in the United States and throughout the world. There is no cure for COPD. The damage to your airways and lungs cannot be reversed, but there are things you can do to feel better and slow the damage. COPD is not contagious—you cannot catch it from someone else. What Is Cystic Fibrosis?Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky. The mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs. The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully. As a result:
The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency. CF can also cause infertility (mostly in men). The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults. Respiratory failure is the most common cause of death in people with CF. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old. Research continues to look for:
What Are Pleurisy and Other Disorders of the Pleura?Pleurisy (PLUR-is-see) is inflammation (swelling) of the pleura. The pleura is a large, thin sheet of tissue (membrane) that wraps around the outside of your lungs and lines the inside of your chest cavity. Between the layer of the pleura that wraps around your lungs and the layer that lines your chest cavity is a very thin space. This is called the pleural space. Normally it's filled with a small amount of fluid—about 4 teaspoons full. The fluid helps the two layers of the pleura glide smoothly past each other as your lungs breathe air in and out. Pleurisy occurs when the two layers of the pleura become red and inflamed. Then they rub against each other every time your lungs expand to breathe in air. This can cause sharp pain with breathing. Infections like pneumonia are the most common cause of swelling, or inflammation, of the pleura and pleurisy. Other Disorders of the PleuraPleural EffusionIn some cases of pleurisy, excess fluid builds up in the pleural space. This is called a pleural effusion. The buildup of fluid usually forces the two layers of the pleura apart so they don't rub against each other when you breathe. This can relieve your pain. However, a large amount of extra fluid can push the pleura against your lung until the lung, or a part of it, collapses. This can make it hard for you to breathe. In some cases of pleural effusion, the extra fluid gets infected and turns into an abscess. This is called an empyema (em-pi-E-ma). You can develop a pleural effusion if you don't have pleurisy. For example, pneumonia, heart failure, cancer, or a pulmonary embolism can lead to a pleural effusion. PneumothoraxAir or gas also can build up in the pleural space. This is called a pneumothorax (noo-mo-THOR-aks). It can result from acute lung injury or a lung disease like emphysema. Lung procedures, like surgery, drainage of fluid with a needle, examination of the lung from the inside with a light and a camera, or mechanical ventilation, also can cause it. The most common symptom is sudden pain in one side of the lung and shortness of breath. A pneumothorax also can put pressure on the lung and cause it to collapse. If the pneumothorax is small, it may go away on its own. If it's large, you may need to have a tube placed through your skin and chest wall into the pleural space to remove the air. HemothoraxBlood also can collect in the pleural space. This is called hemothorax (he-mo-THOR-aks). The most common cause is injury to your chest from blunt force or chest or heart surgery. Hemothorax also can occur in people with lung or pleural cancer. Hemothorax can put pressure on the lung and force it to collapse. It also can cause shock, a state in which not enough blood and oxygen reach important organs in the body. OutlookPleurisy and other disorders of the pleura can be serious, depending on what caused the inflammation in the pleura. If the condition that caused the pleurisy or other pleural disorders isn't too serious and is diagnosed and treated early, you usually can expect a full recovery. What Is Idiopathic Pulmonary Fibrosis?Pulmonary (PULL-mun-ary) fibrosis is a condition in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The development of the scarred tissue is called fibrosis. As the lung tissue becomes thicker, your lungs lose their ability to move oxygen into your bloodstream, and your brain and other organs don’t receive the oxygen they need. In some cases, doctors can find out what is causing the fibrosis. But in most cases, they can’t find a cause. They call these cases idiopathic pulmonary fibrosis (IPF). IPF is a serious condition. About 200,000 Americans have it. About 50,000 new cases are diagnosed each year. IPF affects mostly people 50 to 75 years of age. IPF varies from person to person. In some people, the lung tissue quickly becomes thick and stiff. In others, the process is much slower, and in some people, the condition stays the same for years. There is no cure for IPF yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes include:
Some people may be more likely to develop IPF because of their genes (the basic units of heredity). If more than one member of your family has IPF, the condition is called familial idiopathic pulmonary fibrosis. Today, scientists are beginning to understand more about what causes IPF, and they can diagnose it more quickly. They also are studying several medicines that may slow the progress of the disease. These efforts should lead to longer and better quality lives for people with IPF. What Is LAM?LAM, or lymphangioleiomyomatosis (lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), is a rare lung disease that mostly affects women in their mid-forties. In LAM, an unusual type of cell begins to grow out of control throughout your body, including in the lungs, lymph nodes and vessels, and kidneys. Over time, these LAM cells form cysts and clusters of cells, which grow throughout the lungs and slowly block the airways. They also destroy the normal lung tissue and replace it with cysts. As a result, air cannot move freely in and out of the lungs, and the lungs cannot supply enough oxygen to the body’s other organs. More than 1 out of every 3 people with LAM also develops growths called angiomyolipomas (AN-je-o-my-o-li-PO-mas), or AMLs, in their kidneys. People with LAM also may develop:
There currently is no cure for LAM. The most common cause of death from LAM is respiratory failure. There are two forms of LAM:
The term “lymphangioleiomyomatosis” comes from the Greek. “Lymph” and “angio” refer to the lymph vessels, and “leiomyomas” refers to the type of cells involved in LAM. Doctors have learned a lot about LAM in recent years. They are now able to diagnose the condition earlier and provide support services that improve patients’ quality of life. Not too long ago, doctors thought that the life expectancy for women with LAM was less than 10 years following diagnosis. We now know that some patients with LAM may survive for more than 20 years. What Is Pneumonia?Pneumonia (nu-MO-ne-ah) is an infection in one or both of the lungs. Many small germs, such as bacteria, viruses, and fungi, can cause pneumonia. The infection causes your lungs’ air sacs, called alveoli (al-VEE-uhl-eye), to become inflamed. The air sacs may fill up with fluid or pus, causing symptoms such as a cough (with phlegm), fever, chills, and trouble breathing. OverviewPneumonia and its symptoms can vary from mild to severe. Many factors affect how serious pneumonia is, such as the type of germ causing the infection and your age and overall health. Pneumonia tends to be more serious for:
What Is Pulmonary Embolism?A pulmonary embolism (PULL-mun-ary EM-bo-lizm), or PE, is a sudden blockage in a lung artery, usually due to a blood clot that traveled to the lung from a vein in the leg. 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. PE is a serious condition that can cause:
If the blood clot is large, or if there are many clots, PE can cause death. OverviewIn most cases, PE 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. These clots can break free, travel through the bloodstream to the lungs, and block an artery. This is unlike clots in the veins close the skin's surface, which remain in place and do not cause PE. OutlookAt least 100,000 cases of PE occur each year in the United States. PE is the third most common cause of death in hospitalized patients. If left untreated, about 30 percent of patients with PE will die. Most of those who die do so within the first few hours of the event. What Is Pulmonary Arterial Hypertension?Pulmonary (PULL-mun-ary) arterial hypertension (PAH) is continuous high blood pressure in the pulmonary artery. The average blood pressure in a normal pulmonary artery is about 14 mmHg when the person is resting. In PAH, the average is usually greater than 25 mmHg. PAH is a serious condition for which there are treatments but no cure. Treatment benefits many patients. The pulmonary arteries are the blood vessels that carry oxygen-poor blood from the right ventricle (VEN-trih-kul) in the heart to the small arteries in the lungs. In PAH, three types of changes may occur in the pulmonary arteries:
There is less room for the blood to flow through these narrower arteries. The arteries may also stiffen. Over time, some of the arteries may become completely blocked. The narrowing of the pulmonary arteries causes the right side of heart to work harder to pump blood through the lungs. Over time, the heart muscle weakens and loses its ability to pump enough blood for the body's needs. This is called right heart failure. Heart failure is the most common cause of death in people with PAH. There are two types of PAH:
About 300 new cases of PPAH are diagnosed in the United States each year. SPAH is much more common. Doctors have learned a lot about PAH in recent years. More treatments are now available. Researchers are also studying several promising new treatments that may prolong lives as well as improve the quality of life for people living with PAH. What Is Sarcoidosis?Sarcoidosis (sar"koi-do'sis) involves inflammation that produces tiny lumps of cells in various organs in your body. The lumps are called granulomas (gran"u-lo'mahs) because they look like grains of sugar or sand. They are very small and can be seen only with a microscope. These tiny granulomas can grow and clump together, making many large and small groups of lumps. If many granulomas form in an organ, they can affect how the organ works. This can cause symptoms of sarcoidosis. Sarcoidosis can occur in almost any part of your body, although it usually affects some organs more than others. It usually starts in one of two places:
Sarcoidosis also often affects your:
Less often, sarcoidosis affects your:
Rarely, sarcoidosis affects other organs, including your:
Sarcoidosis almost always occurs in more than one organ at a time. Sarcoidosis has an active and a nonactive phase:
The course of the disease varies greatly among people.
Changes in sarcoidosis usually occur slowly (e.g., over months). Sarcoidosis does not usually cause sudden illness. However, some symptoms may occur suddenly. They include:
In some serious cases in which vital organs are affected, sarcoidosis can result in death. Sarcoidosis is not a form of cancer. There is no known way to prevent sarcoidosis. Sarcoidosis was once thought to be an uncommon condition. It's now known to affect tens of thousands of people throughout the United States. Because many people who have sarcoidosis have no symptoms, it's hard to know how many people have the condition. Sarcoidosis was identified in the late 1860s. Since then, scientists have developed better tests to diagnose it and made advances in treating it. What Is Thoracentesis?Thoracentesis (THOR-a-sen-TE-sis) is a procedure to remove excess fluid in the space between the lungs and the chest wall. This space is called the pleural space. Normally, the pleural space is filled with a small amount of fluid—about 4 teaspoons full. But some conditions, such as heart failure, lung infections, and tumors, can cause more fluid to build up. When this happens, it’s called a pleural effusion. A lot of extra fluid can press on the lungs, making it hard to breathe. OverviewThoracentesis is done to find the cause of a pleural effusion. It also may be done to help you breathe easier. During the procedure, your doctor inserts a thin needle or plastic tube into the pleural space and draws out the excess fluid. Usually, doctors take only the amount of fluid needed to find the cause of the pleural effusion. However, if there's a lot of fluid, they may take more. This helps the lungs expand and take in more air, which allows you to breathe easier. After the fluid is removed from your chest, it's sent for testing. Once the cause of the pleural effusion is known, your doctor will plan treatment. For example, if an infection is causing the excess fluid, you may be given antibiotics to fight the infection. If the cause is heart failure, you will be treated for that condition. Thoracentesis usually takes 10 to 15 minutes. It may take longer if there's a lot of fluid in the pleural space. You will be watched for up to a few hours after the procedure for complications. OutlookThe procedure usually doesn't cause serious problems, but some risks are involved. These include pneumothorax (noo-mo-THOR-aks), or collapsed lung; pain, bleeding, bruising, or infection where the needle or tube was inserted; and liver or spleen injury (very rare). Most of these complications get better on their own, or they're easily treated.
Credits: Information on this page is provided with permission by the National Institute or Health; National Heart, Lung and Blood Institute.
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Last Modified: 05/14/08
