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Idiopathic Pulmonary Fibrosis

more about Idiopathic Pulmonary Fibrosis


Fibrosing alveolitis



Normal

Abnormal
  • This is a disease of the lungs, in which the lung tissue becomes fibrosed (scarred and string-like), interfering with the body's ability to properly exchange oxygen from the air. It usually occurs when one is in their 50s-60s, and is a progressively worsening lung disease.

  • Slowly worsening cough and shortness of breath over months or years

  • Unknown

  • Examination

- Fine crackles in lungs may be present

  • Chest X-Ray -- shows signs of interstitial lung disease
  • CT scan shows parenchymal lung fibrosis
  • Pulmonary function test shows a restrictive pattern
  • ANA and Rheumatoid factor may be positive
  • Bronchiolar lavage and/or lung biopsy will make a definitive diagnosis

  • Supplemental oxygen
  • Corticosteroids if symptoms are progressing or lung function deteriorating.
  • Cyclophosphamide and Azathioprine may be helpful.
  • Interferon beta 1a is a new treatment being tried in some.
  • Heart-lung and single-lung transplantation may be appropriate for certain individuals with end-stage disease.




more about Idiopathic Pulmonary Fibrosis


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