Idiopathic Pulmonary Fibrosis (IPF) is a disease of inflammation that results in scarring or fibrosis, of the lungs. In time, this fibrosis can build up to the point where the lungs are unable to provide oxygen to the tissues of the body. Doctors use the word "idiopathic" (from the Greek "idio" meaning "peculiar" or "unusual" and "pathy" meaning "illness") to describe the disease, because the cause of IPF is unknown. Currently, researchers believe that IPF may result from either an autoimmune disorder, a condition in which the body's immune system attacks its own tissues, or the aftereffects of an infection, most likely a virus. Whatever the trigger is for IPF, it appears to set off a series of events in which the inflammation and immune activity in the lungs--and, eventually, the fibrosis processes, too--become uncontrollable. In a few cases, heredity appears to play a part, possibly making some individuals more likely than others to get IPF. In studies of patients with IPF, the average survival rate has been found to be 4 to 6 years after diagnosis. Those who develop idiopathic pulmonary fibrosis at a young age seem to have a longer survival.

The exact number of people who develop idiopathic pulmonary fibrosis each year is not known. It is known, however, that equal numbers of men and women get the illness and that most cases of IPF are diagnosed when the patients are between the ages of 40 and 70.

Early symptoms of idiopathic pulmonary fibrosis are usually similar to those of other lung diseases. Very often, for example, patients suffer from a dry cough and dyspnea (shortness of breath). As the disease progresses, dyspnea becomes the major problem. Day-to-day activities such as climbing stairs, walking short distances, dressing, and even talking on the phone and eating become more difficult and sometimes nearly impossible. Enlargement (clubbing) of the fingertips may develop. The patient may also become less able to fight infection. In advanced stages of the illness, the patient may need oxygen all the time. IPF can lead to death. Often the immediate cause is respiratory failure due to hypoxemia, right-heart failure, a heart attack, blood clot (embolism) in the lungs, stroke, or lung infection brought on by the disease.

Although the course of idiopathic pulmonary fibrosis varies greatly from person to person, the disease usually develops slowly, sometimes over years. The early stages are marked by alveolitis, an inflammation of the air sacs called alveoli, in the lungs. The job of the air sacs is to allow the transfer of oxygen from the lungs into the blood and the elimination of carbon dioxide from the lungs and out of the body. As IPF progresses, the alveoli become damaged and scarred, thus stiffening the lungs. The stiffening makes breathing difficult and brings on a feeling of breathlessness (dyspnea), especially during activities that require extra effort. In addition, scarring of the alveoli reduces the ability of the lungs to transfer oxygen. The resulting lack of oxygen in the blood (hypoxemia) may cause increases in the pressure inside the blood vessels of the lungs, a situation known as pulmonary hypertension. The high blood pressure in the lungs then puts a strain on the right ventricle, the lower right side of the heart, which pumps the oxygen-poor blood into the lungs.

The first suspicion that a person may have idiopathic pulmonary fibrosis is usually based on the patient's symptoms and medical history. The doctor will try to confirm or rule out any suspicion by ordering one or more of the following tests: chest x ray (CXR), computed tomography (CT or CAT Scan), blood tests, pulmonary function tests (PFT's). Even if some or all of the results from such tests are abnormal, they are rarely sufficient to make a specific diagnosis of IPF. The only way the doctor can confirm a diagnosis of IPF is by examining the lung tissue; such tissue is usually obtained by bronchoscopy or an open lung biopsy. More information about these tests can be found in a different patient information sheet.

The best chance of slowing the progress of IPF is by treatment as soon as possible. Most IPF patients require treatment throughout life, usually under the guidance of a lung specialist. Some major medical centers and large teaching hospitals do research on the disease and provide consultation and treatment to patients. Treatment for idiopathic pulmonary fibrosis may vary a great deal. It depends on many things, including the age of the patient and stage of the disease. The aim of treatment is to reduce the inflammation or fibrosis of the alveoli and stop the abnormal process that ends in fibrosis. Once scar tissue has formed in the lung, it cannot be returned to normal.

Drugs are the primary way that IPF is treated. They are usually prescribed for at least 3 to 6 months. This gives the doctor time to see if a particular treatment is effective. A combination of tests is used to monitor how well a particular drug is working. The dose may have to be adjusted so that the medicine gives the best possible results with the least side effects. Most side effects are reduced when the dose is made smaller or the drug is stopped. Commonly used drugs are prednisone and cytoxan. Oxygen administration and, in special cases, transplantation of the lung are other choices. Recently, Interferon Gamma has been evaluated for IPF and appears to be promising. A current study evaluating this therapy is in a Phase III trial, and the results are expected in November 2002. More information about treatment can be found in a different information sheet.

Many IPF patients, particularly those in the early stages of the disease, respond to drug treatment and can continue to go about most of their normal activities, including working. Some patients with advanced IPF need to carry oxygen with them. In addition to getting proper treatment, IPF patients can help themselves by following the same sensible health measures that everyone should observe. These include eating a healthy diet, maintaining proper weight, exercising regularly, and getting enough rest. Above all, IPF patients should not smoke. Pregnancy is not advisable because the illness puts an extra load on the heart and lungs. As with many chronic illnesses, emotional support and psychological counseling can be of much help to the patient. Most doctors and patients agree that it is important for both patient and family to be as informed as possible about IPF. In this way, everyone involved can understand the illness and apply that information to what is happening in his or her own life.

Some people with idiopathic pulmonary fibrosis may be eligible to participate in an experimental clinical trial at various locations around the country. Your doctor can give you more information about possible clinical trials. Participants must meet the specific requirements for any particular study, and often have to travel.

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