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Idiopathic pulmonary fibrosis

  • Alternative Names

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

    • 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)

    Signs 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) 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).

    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
    • Surgical lung biopsy
    • Tests for connective tissue diseases such as rheumatoid arthritis, lupus, or scleroderma