Idiopathic pulmonary fibrosis

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Clubbing

Respiratory system

Spirometry

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

• 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)

The health care provider will perform a physical exam and ask questions about your medical history.

Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) 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
• CT - chest
• Pulmonary function tests
• Bronchoscopy with transbronchial lung biopsy
• Lung biopsy
• Measurements of blood oxygen level
• Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma

Review Date: 05/03/2006
Reviewed By: David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.