Idiopathic diffuse interstitial pulmonary fibrosis
Key points
- Idiopathic pulmonary fibrosis involves scarring or thickening of tissues deep in the lung without a known cause.
- Idiopathic pulmonary fibrosis is a disease of the lower respiratory tract that damages the air sacs (alveoli) and leads to reduced transfer of oxygen to the blood.
- In addition to a physical examination, your health care provider will take a careful history in order to exlude other similar diseases.
- No known cure exists for idiopathic pulmonary fibrosis.
Alternative names
Idiopathic pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Definition
Idiopathic pulmonary fibrosis involves scarring or thickening of tissues deep in the lung without a known cause.
Causes, incidence, and risk factors
Idiopathic pulmonary fibrosis is a disease of the lower respiratory tract that damages the air sacs (alveoli) and leads to reduced transfer of oxygen to the blood. It causes widespread scarring of the lung.
The condition is believed to result from an inflammatory response to an unknown agent - "idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old.
Symptoms
- Shortness of breath on exertion that lasts for months or years and eventually is present at rest
- Decreased tolerance for activity
- Cough (usually dry)
- Chest pain (occasionally)
Signs and tests
In addition to a physical examination, your health care provider will take a careful history in order to exlude other similar diseases.
Patients with idiopathic pulmonary fibrosis have dry, Velcro-like breath sounds called crackles. Patients with advanced disease may have cyanosis (blueness around the mouth or in the fingernails due to low oxygen).
Examination of the fingers and toes may reveal clubbing (abnormal enlargement of the fingernail bases).
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
- Chest X-ray
- High-resolution CT scan of the chest
- Pulmonary function tests
- Bronchoscopy with transbronchial lung biopsy
- Surgical lung biopsy
- Measurement of blood oxygen level at rest or during exertion
- Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma
Treatment
No known cure exists for idiopathic pulmonary fibrosis. Medications such as corticosteroids and cytotoxic drugs may be given to suppress inflammation, but these treatments are usually unsuccessful. Oxygen is given to patients who have low blood oxygen levels.
Written and revised by
Gerald Hurowitz, M.D.
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