Idiopathic pulmonary fibrosis Email this page to a friendShare on facebook Share on twitterBookmark & SharePrinter-friendly version Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Causes No one knows what causes idiopathic pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening may make it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time. The condition is believed to be due to an inflammatory response to an unknown substance or injury. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old.
Symptoms
Chest pain (occasionally)
Cough (usually dry)
Decreased tolerance for activity
Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
Exams and Tests The health care provider will perform a physical exam and ask questions about your medical history. Your doctor will ask whether you have been exposed to asbestos and if you have been a smoker. Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis cyanosis)) around the mouth or in the fingernails due to low oxygen. Examination of the fingers and toes may show abnormal enlargement of the fingernail bases ( clubbing clubbing)). Tests that help diagnose idiopathic pulmonary fibrosis include the following:
Bronchoscopy with transbronchial lung biopsy
Chest CT scan
Chest x-ray
Measurements of blood oxygen level
Pulmonary function tests
Six-minute walk test
Surgical lung biopsy
Tests for connective tissue diseases such as rheumatoid arthritis, arthritis, lupus, or scleroderma or scleroderma
Treatment No known cure exists for idiopathic pulmonary fibrosis. Unfortunately, no medication has been shown to improve the outcome of patients with this condition.
For some people, medications such as corticosteroids and cytotoxic drugs may help reduce swelling (inflammation). However, these drugs might also increase the risk of death.
Other new treatments that have been shown to help some people with idiopathic pulmonary fibrosis are being studied.
Patients with low blood oxygen levels may need oxygen. oxygen.
Lung rehabilitation will not cure the disease, but it can help maintain exercise capacity (the ability to exercise without breathing difficulty).
You can make home and lifestyle changes to manage breathing symptoms. Anyone who smokes should stop right away. Some patients with advanced pulmonary fibrosis may need a lung transplant.
Support Groups You can ease the stress of illness by joining a support group where members share common experiences and problems. The following organizations are good resources for information on lung disease:
American Lung Association - www.lungusa.org
National Heart, Lung, and Blood Institute - www.nhlbi.nih.gov
Outlook (Prognosis) Some patients may improve or stay stable for a long time when they are treated with corticosteroids or cytotoxic drugs. However, in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly. When breathing symptoms become more severe, discuss treatments that prolong life, health care agents, andadvanced care directives with your health care provider.
Possible Complications
Abnormally high levels of red blood cells due to low blood oxygen levels
Collapsed lung
High blood pressure in the arteries of the lungs
Respiratory failure
When to Contact a Medical Professional Call your health care provider if your breathing is:
Getting harder
Faster than before
Shallow, and you cannot get a deep breath
Also call your health care provider if:
You need to lean forward when sitting to breathe comfortably.
You are using muscles around your ribs to help you breathe.
You are having headaches more often.
You feel sleepy or confused.
You have a fever.
You are coughing up dark mucus.
Your fingertips, or the skin around your fingernails is blue.
