Question: What is (are) Idiopathic Pulmonary Fibrosis ?
Answer: Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a disease in which tissue deep in your lungs becomes thick and stiff, or scarred, over time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can't properly move oxygen into your bloodstream. As a result, your brain and other organs don't get the oxygen they need. (For more information, go to the "How the Lungs Work" section of this article.) Sometimes doctors can find out what's causing fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic (id-ee-o-PATH-ick) pulmonary fibrosis (IPF). IPF is a serious disease that usually affects middle-aged and older adults. IPF varies from person to person. In some people, fibrosis happens quickly. In others, the process is much slower. In some people, the disease stays the same for years. IPF has no cure 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 of death include pulmonary hypertension (HI-per-TEN-shun), heart failure, pulmonary embolism (EM-bo-lizm), pneumonia (nu-MO-ne-ah), and lung cancer. Genetics may play a role in causing IPF. If more than one member of your family has IPF, the disease is called familial IPF. Research has helped doctors learn more about IPF. As a result, they can more quickly diagnose the disease now than in the past. Also, researchers are studying several medicines that may slow the progress of IPF. These efforts may improve the lifespan and quality of life for people who have the disease.
Source: NHLBI

Question: What causes Idiopathic Pulmonary Fibrosis ?
Answer: Sometimes doctors can find out what is causing pulmonary fibrosis (lung scarring). For example, exposure to environmental pollutants and certain medicines can cause the disease. Environmental pollutants include inorganic dust (silica and hard metal dusts) and organic dust (bacteria and animal proteins). Medicines that are known to cause pulmonary fibrosis in some people include nitrofurantoin (an antibiotic), amiodarone (a heart medicine), methotrexate and bleomycin (both chemotherapy medicines), and many other medicines. In most cases, however, the cause of lung scarring isnt known. These cases are called idiopathic pulmonary fibrosis (IPF). With IPF, doctors think that something inside or outside of the lungs attacks them again and again over time. These attacks injure the lungs and scar the tissue inside and between the air sacs. This makes it harder for oxygen to pass through the air sac walls into the bloodstream. The following factors may increase your risk of IPF: Cigarette smoking Viral infections, including Epstein-Barr virus (which causes mononucleosis), influenza A virus, hepatitis C virus, HIV, and herpes virus 6 Genetics also may play a role in causing IPF. Some families have at least two members who have IPF. Researchers have found that 9 out of 10 people who have IPF also have gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may regularly breathe in tiny drops of acid from their stomachs. The acid can injure their lungs and lead to IPF. More research is needed to confirm this theory.
Source: NHLBI

Question: What are the symptoms of Idiopathic Pulmonary Fibrosis ?
Answer: The signs and symptoms of idiopathic pulmonary fibrosis (IPF) develop over time. They may not even begin to appear until the disease has done serious damage to your lungs. Once they occur, they're likely to get worse over time. The most common signs and symptoms are: Shortness of breath. This usually is the main symptom of IPF. At first, you may be short of breath only during exercise. Over time, you'll likely feel breathless even at rest. A dry, hacking cough that doesn't get better. Over time, you may have repeated bouts of coughing that you can't control. Other signs and symptoms that you may develop over time include: Rapid, shallow breathing Gradual, unintended weight loss Fatigue (tiredness) or malaise (a general feeling of being unwell) Aching muscles and joints Clubbing, which is the widening and rounding of the tips of the fingers or toes Clubbing IPF may lead to other medical problems, including a collapsed lung, lung infections, blood clots in the lungs, and lung cancer. As the disease worsens, you may develop other potentially life-threatening conditions, including respiratory failure, pulmonary hypertension, and heart failure.
Source: NHLBI

Question: How to diagnose Idiopathic Pulmonary Fibrosis ?
Answer: Idiopathic pulmonary fibrosis (IPF) causes the same kind of scarring and symptoms as some other lung diseases. This makes it hard to diagnose. Seeking medical help as soon as you have symptoms is important. If possible, seek care from a pulmonologist. This is a doctor who specializes in diagnosing and treating lung problems. Your doctor will diagnose IPF based on your medical history, a physical exam, and test results. Tests can help rule out other causes of your symptoms and show how badly your lungs are damaged. Medical History Your doctor may ask about: Your age Your history of smoking Things in the air at your job or elsewhere that could irritate your lungs Your hobbies Your history of legal and illegal drug use Other medical conditions that you have Your family's medical history How long you've had symptoms Diagnostic Tests No single test can diagnose IPF. Your doctor may recommend several of the following tests. Chest X Ray A chest x ray is a painless test that creates a picture of the structures in your chest, such as your heart and lungs. This test can show shadows that suggest scar tissue. However, many people who have IPF have normal chest x rays at the time they're diagnosed. High-Resolution Computed Tomography A high-resolution computed tomography scan, or HRCT scan, is an x ray that provides sharper and more detailed pictures than a standard chest x ray. HRCT can show scar tissue and how much lung damage you have. This test can help your doctor spot IPF at an early stage or rule it out. HRCT also can help your doctor decide how likely you are to respond to treatment. Lung Function Tests Your doctor may suggest a breathing test called spirometry(spi-ROM-eh-tree) to find out how much lung damage you have. This test measures how much air you can blow out of your lungs after taking a deep breath. Spirometry also measures how fast you can breathe the air out. If you have a lot of lung scarring, you won't be able to breathe out a normal amount of air. Pulse Oximetry For this test, your doctor attaches a small sensor to your finger or ear. The sensor uses light to estimate how much oxygen is in your blood. Arterial Blood Gas Test For this test, your doctor takes a blood sample from an artery, usually in your wrist. The sample is sent to a laboratory, where its oxygen and carbon dioxide levels are measured. This test is more accurate than pulse oximetry. The blood sample also can be tested to see whether an infection is causing your symptoms. Skin Test for Tuberculosis For this test, your doctor injects a substance under the top layer of skin on one of your arms. This substance reacts to tuberculosis (TB). If you have a positive reaction, a small hard lump will develop at the injection site 48 to 72 hours after the test. This test is done to rule out TB. Exercise Testing Exercise testing shows how well your lungs move oxygen and carbon dioxide in and out of your bloodstream when you're active. During this test, you walk or pedal on an exercise machine for a few minutes. An EKG(electrocardiogram) checks your heart rate, a blood pressure cuff checks your blood pressure, and a pulse oximeter shows how much oxygen is in your blood. Your doctor may place a catheter (a flexible tube) in an artery in one of your arms to draw blood samples. These samples will provide a more precise measure of the oxygen and carbon dioxide levels in your blood. Your doctor also may ask you to breathe into a tube that measures oxygen and carbon dioxide levels in your blood. Lung Biopsy For a lung biopsy, your doctor will take samples of lung tissue from several places in your lungs. The samples are examined under a microscope. A lung biopsy is the best way for your doctor to diagnose IPF. This procedure can help your doctor rule out other conditions, such as sarcoidosis (sar-koy-DO-sis), cancer, or infection. Lung biopsy also can show your doctor how far your disease has advanced. Doctors use several procedures to get lung tissue samples. Video-assisted thoracoscopy (thor-ah-KOS-ko-pee). This is the most common procedure used to get lung tissue samples. Your doctor inserts a small tube with an attached light and camera into your chest through small cuts between your ribs. The tube is called an endoscope. The endoscope provides a video image of the lungs and allows your doctor to collect tissue samples. This procedure must be done in a hospital. You'll be given medicine to make you sleep during the procedure. Bronchoscopy (bron-KOS-ko-pee). For a bronchoscopy, your doctor passes a thin, flexible tube through your nose or mouth, down your throat, and into your airways. At the tube's tip are a light and mini-camera. They allow your doctor to see your windpipe and airways. Your doctor then inserts a forceps through the tube to collect tissue samples. You'll be given medicine to help you relax during the procedure. Bronchoalveolar lavage (BRONG-ko-al-VE-o-lar lah-VAHZH). During bronchoscopy, your doctor may inject a small amount of salt water (saline) through the tube into your lungs. This fluid washes the lungs and helps bring up cells from the area around the air sacs. These cells are examined under a microscope. Thoracotomy (thor-ah-KOT-o-me). For this procedure, your doctor removes a few small pieces of lung tissue through a cut in the chest wall between your ribs. Thoracotomy is done in a hospital. You'll be given medicine to make you sleep during the procedure.
Source: NHLBI