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Idiopathic Pulmonary Fibrosis (IPF)

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 properly provide oxygen to the tissues of the body.

Doctors use the word "idiopathic" 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.

What Are the Symptoms of IPF?

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 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.

What is the Course of IPF?

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.

Can IPF be Treated?

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 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.

How is IPF Treated?

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.

A corticosteroid, prednisone, is the most common drug given to patients with idiopathic pulmonary fibrosis. About 25 to 35 percent of all patients respond favorably to this medicine. No one knows exactly how corticosteroids work or why some patients do well on prednisone while others do not. Patients take prednisone by mouth every morning, starting with a high dose for the first 4 to 8 weeks. As they improve, they gradually take smaller amounts. Changes in mood are one of the more common side effects of prednisone; most patients, however, can handle the mood changes-- anxiety, depression, or sleeplessness--once they know what is causing the problem. A less common side effect is a rise in blood-sugar levels.

Cyclophosphamide, also referred to as cytoxan, may be taken together with prednisone, or instead of it. Like prednisone, cytoxan is swallowed each day. One of the more serious side effects of cyclophosphamide is leukopenia, a condition in which the number of white blood cells drops to a dangerously low level. Leukopenia can be controlled by regularly checking the blood count and adjusting the dose of cytoxan if necessary.

Azathioprine, penicillamine, chlorambucil, vincristine sulfate, and colchicine have been used in a few patients with idiopathic pulmonary fibrosis. Their effectiveness in treating IPF, however, has not been adequately tested.

In addition to treatment with medicine, some patients may need oxygen, especially when blood oxygen becomes low. This treatment helps resupply the blood with oxygen. As a result, breathlessness is reduced, the patient can be more active, and the severity of pulmonary hypertension decreases.

Regular exercise may be useful for patients with IPF. A daily walk or regular use of a stationary bicycle or treadmill can improve muscle strength and breathing ability and also increase overall strength. If needed, supplemental oxygen should be used; sometimes it is the only way a patient is able to do a reasonable amount of activity.

Lung transplantation, either of both lungs or only one, is an alternative to drug treatment for patients in the severe, final stages of IPF. It is most often performed in patients under 60 years of age who do not respond to any form of treatment. The 1-year survival rate is approximately 60 percent.

How Will IPF Affect a Patient's Lifestyle?

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.

Note: The source of the above content is a public domain document: NIH Publication No. 93-2997, dated Feb 93. The complete document can be found at IPF.