Idiopathic pulmonary fibrosis

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Spirometry

Sarcoid, stage IV - chest X-ray

Idiopathic pulmonary fibrosis

Alternative Names:

Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
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:

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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