Initial Symptoms
Individuals with IPF gradually begin to experience some or all of the following symptoms or signs of illness:
- Shortness of breath during or after physical activity
- Spasmodic, dry cough
- Gradual, unintended weight loss
- Fatigue or malaise
- Clubbing, or enlargement of the ends of the fingers (or sometimes the toes) due to a buildup of tissue in the fingertips (click here to see a picture of clubbing)
Disease Progression
IPF affects each person differently and progresses at varying rates. Generally, the patient's breathlessness becomes worse over time. Daily activities (such as walking or climbing stairs) become more difficult.
In addition:
- The patient may require supplemental oxygen to meet the needs of the body.
- Advanced IPF may make it difficult for a person to fight infection.
- IPF causes a lack of oxygen in the blood. This condition (called hypoxemia) may lead to high blood pressure in the lungs (pulmonary hypertension) and put a strain on the heart that might lead to heart dysfunction (called right heart failure, or cor pulmonale).
- IPF has also been associated with these potentially life-threatening conditions: heart attack, respiratory failure, stroke, blood clot in the lungs (pulmonary embolism), or lung infection.
Diagnosis
Because IPF has symptoms and an underlying pattern of scarring that are similar to other lung disorders, it can be difficult to diagnose. It is particularly important for your doctor to be sure that there are no occupational or environmental causes of the lung disease. Until recently, the medical community had no agreed-upon standards for the diagnosis of IPF. Consequently, other related diseases may have been mistakenly diagnosed as IPF. With new diagnostic standards defined by the American Thoracic Society (ATS), European Respiratory Society (ERS), and American College of Chest Physicians (ACCP) now in place, the recognition and management of IPF should be substantially improved. (For a copy of the recently released International Consensus Statement on idiopathic pulmonary fibrosis, click here.)
To diagnose IPF, your doctor will take a complete medical history, with particular emphasis on past occupational or environmental exposures, perform a thorough physical examination, and conduct a number of tests. During your physical exam, the doctor will use a stethoscope to listen to your chest to try to determine if your lungs produce any abnormal sounds when you breathe. He or she may then order one or more of the following diagnostic tests or procedures.
| Diagnostic Procedure |
Description |
Purpose |
| Chest imaging |
Use of radiologic machines to take pictures (x-rays or CT scans) of the lungs. |
To view lung structures, look for scar tissue, and assess patterns of scarring. |
| Pulmonary function tests |
A series of breathing tests that measure your capacity to move air in and out, to take a deep breath, or to exchange oxygen from air to blood. |
To measure the size and effectiveness of your lungs compared to what would be expected for someone your age. Also, to determine the degree of impairment and the progress of the disease over time. |
| Arterial blood gas test |
A measurement of oxygen levels in blood taken from an artery. |
To determine how well the lungs are absorbing oxygen and getting rid of the carbon dioxide in your blood. |
| Exercise test |
A test in which the patient is monitored while using a treadmill or stationary bicycle. |
To measure how well the lungs and heart respond to physical activity. |
Bronchoalveolar lavage
(BAL) |
A "lung-washing" procedure conducted through a flexible tube (bronchoscope) inserted into the airways through the nose or mouth; fluid (salt water) is instilled into the lungs and then removed for inspection. |
To examine cells and look for signs of inflammation in the lungs. |
| Lung biopsy |
A procedure in which a small piece of your lung tissue is obtained through a bronchoscope (see BAL, above) or by a surgeon through a small incision between the ribs (open-lung biopsy or video-assisted thoracotomy). |
To obtain a sample of lung tissue for direct examination. |
The recent International Consensus Statement on the diagnosis and treatment of IPF recommends a surgical lung biopsy for most patients because it is the most conclusive way to determine whether a patient has IPF. The lung tissue of a patient with IPF displays certain characteristics and patterns not found in other diseases. Your doctor will determine if this procedure is critical for your diagnosis.
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