2015 Top 10 Medical Innovationk: New Drugs for Idiopathic Pulmonary Fibrosis

What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a progressive disease (gets worse over time) isolated to the lung. It is a type of interstitial lung disease, which is a group of 200 diseases with similar symptoms but different causes. Currently, more than 80,000 adults in the United States have IPF, and more than 30,000 new cases are diagnosed each year.

In IPF, lung tissue becomes scarred and changes the lung's ability to function normally. The scarring typically starts at the edges of the lungs and advances towards the center of the lungs. Typically, mild scarring occurs first, but over months to years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body. Unfortunately, IPF is a disabling disease without a known cure and with few treatment options.

What causes idiopathic pulmonary fibrosis?

The cause of IPF is unknown, but the following are possible risk factors for the disease:

  • Working around dust or fumes. Farmers, ranchers, hairdressers, stone cutters/polishers, and metal workers face a moderately increased risk of developing the disease because of exposure to these dusts or occupational fumes.
  • Age between 40 and 70 (approximately 2/3 of patients are older than 60 at the time of diagnosis)
  • History of smoking
  • Male gender (more men are diagnosed with the disease than women)
  • Genetics (inheriting the disease from parents)

What are the symptoms of idiopathic pulmonary fibrosis?

Symptoms of IPF usually develop gradually and may not be noticed until the disease is well-established. IPF affects each person differently and the disease progresses at varying rates. Symptoms include:

  • Dry cough
  • Shortness of breath, especially during or after physical activity
  • Fatigue
  • Weight loss
  • Clubbing, a widening and rounding of the tips of the fingers
  • Swelling of the legs

How is idiopathic pulmonary fibrosis diagnosed?

To diagnose IPF, the doctor will take a thorough medical history to rule out other lung-related illnesses or medical causes, and then perform a physical examination. The physical exam is not specific enough to make the diagnosis of IPF, but it can identify some telltale signs. The doctor will also listen to the patient’s chest to determine if the lungs are producing any abnormal sounds when the patient breathes. The doctor may then order one or more of the diagnostic tests or procedures shown in the chart. To make a diagnosis of IPF requires a CT scan of the chest, and sometimes a lung biopsy.

What are the complications of idiopathic pulmonary fibrosis?

When lung tissue becomes scarred, it is less able to deliver needed oxygen to the body. This puts a strain on the right side of the heart, which may lead to high blood pressure in the lungs, a condition known as pulmonary hypertension.

Some patients may have an exacerbation of IPF, which is a sudden increase in lung scarring. During an exacerbation, the patient may cough more, and have more shortness of breath episodes. The causes of an IPF exacerbation are not known, but you should contact your doctor if you suddenly feel worse.

It is also very important to take measures to avoid becoming sick, as it may be more difficult to recover from lung infections. Patients should:

  • Pay close attention to hand washing.
  • Keep up-to-date with immunizations.
  • Avoid direct contact with others who are sick.
Diagnostic tests for idiopathic pulmonary fibrosis
Chest imaging studies X-rays or high-resolution CT scans To rule out other diseases, allow for early (and in some cases definitive) diagnosis, assess extent of disease (changes in bronchial structure, scarring pattern throughout the lung)
Lung biopsy Taking a small sample of lung tissue, usually by a small incision through the ribs with a thoracoscope To directly examine lung tissue to aid in the diagnosis of IPF or other lung diseases.
Pulmonary function test Using a device to measure breathing capacity To measure the degree of damage to lung function and to see if the disease has advanced
Oxygen desaturation study The patient walks for almost six minutes while his or her oxygen level is measured through a probe attached to the finger or the forehead. To determine the need for oxygen and to see if the disease has advanced
Other laboratory tests Autoantibody tests, full blood count, electrolytes, creatinine levels, liver function tests, arterial blood gas To rule out other diseases, monitor changes in body functions over time

How is idiopathic pulmonary fibrosis treated?

There is no cure for IPF. The damage caused by the lung scarring cannot be reversed. In addition, the progression of the disease cannot be predicted or stopped.

There are currently two medications approved for use in IPF. These medications have been shown to slow the scarring and help preserve lung function.

Lung transplant surgery may be considered in some patients, most often patients who are under 70 years old who have advanced disease.

Other treatments for IPF are supportive. Patients may need additional oxygen, which supplied in a small portable oxygen tank, to help them breathe. Patients are also encouraged to enroll in an exercise program known as pulmonary rehabilitation to help improve their breathing. Patients should also receive immunizations for pneumonia and yearly influenza to avoid illness, which can be more severe in people with IPF. It is important to stay active, follow a healthy diet, get plenty of rest, and quit smoking.

Some exciting new treatments are currently under investigation. The Cleveland Clinic is one of 26 medical centers across the United States that excel in the treatment of IPF. These medical centers make up the IPFnet, a network of research centers dedicated to the study of IPF. The network’s goal is to find effective therapies for patients with both the early and advanced stages of this devastating disease. Please consult your doctor if you are interested in taking part in a clinical trial.

How often will I need to see my doctor for followup for idiopathic pulmonary fibrosis?

Your health care providers will work with you to help you maintain the highest possible quality of life. Your first visit will probably take place in your doctor's office. At times, you may need to go to the hospital for tests and treatments. You may need to see your doctor three to four times a year.

Where can I find out about support groups and other resources for idiopathic pulmonary fibrosis?

To learn more about your disease and other support services in your community, contact the following:

American Lung Association
55 W. Wacker Drive, Suite 1150
Chicago, IL 60601
1.800.LUNGUSA or 1.800.548.8252

Pulmonary Fibrosis Association
230 East Ohio Street, Suite 500
Chicago, Illinois 60611

Coalition for Pulmonary Fibrosis
10866 W. Washington Blvd #343
Culver City, CA 90232
(888) 222-8541


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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 2/1/2017…#10959