• 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)

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