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Other Names:
Idiopathic Diffuse Interstitial Pulmonary Fibrosis, IPF, Pulmonary Fibrosis, Cryptogenic Fibrosing Alveolitis, CFA, Fibrosing Alveolitis, Usual Interstitial Pneumonitis, UIP
Pulmonary Fibrosis

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Definition

Pulmonary fibrosis involves scarring and thickening of tissues between the air sacs (alveoli) deep in the lungs. This impairs the ability of the lungs to exchange gases across the blood.

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Causes

  • Inhaling asbestos fibers and silica dust
  • Sarcoidosis of the lung
  • Scleroderma which is a progressive disease that leads to hardening and scarring of the skin and connective tissues
  • Rheumatoid arthritis affecting the lungs leading to:
    • Pleuritis or inflammation of the lining of the lungs, resulting in pain with breathing (pleurisy). Fluid also may accumulate around the lungs between the two layers of the lining membrane (pleural effusion) and cause difficulty breathing.
    • Formation of small lumps or rheumatoid nodules in the lungs.
  • Smoking

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Symptoms

  • Idiopathic or exact cause cannot be determined.
  • Chronic breathing difficulty on activity that eventually progresses to breathlessness present at rest
  • Excessive fatigue
  • Weakness
  • Dry cough
  • Chest pain
  • Cyanosis or blueness around the mouth or in the fingernails due to low oxygen.
  • Clubbing or abnormal enlargement of the fingernail bases

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Implications

Long standing pulmonary fibrosis can lead to respiratory failure and chronic hypoxemia due to low blood oxygen level. Risk of lung cancer for a smoker exposed to asbestos may be as much as 90 times greater than that of a nonsmoker exposed to asbestos or a smoker who's never been exposed to asbestos. The thickened scar tissue may eventually compress or completely block the small blood vessels in the lungs, causing high blood pressure in the arteries of the lungs (pulmonary hypertension). This condition can lead to enlargement and failure of the right ventricle of heart (cor pulmonale). Persistent high blood pressure in the arteries of the lungs requires the heart to work harder to meet the body's demands for oxygen. The heart tries to compensate for this by thickening its walls and dilating the right ventricle to increase the amount of blood it can hold. Over time this compensatory mechanism fails, the right ventricle weakens and fails to perform it's function and handle the extra strain. The pleural membrane may thicken, there may be formation of calcium deposits in the pleura (plaques), and an abnormal accumulation of fluid between the membranes (pleural effusion).

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What You Can Do

  • Oxygen is given to patients who have low blood oxygen levels.
  • Medications like corticosteroids and cytotoxic drugs (such as Azathioprine or Imuran) may be give suppress inflammation.
  • Gamma interferon has shown promising results in treating this disease.
  • Lung transplantation may be indicated for some patients with advanced pulmonary fibrosis.
  • Joining support groups and sharing your experiences and problems of illness helps reduce the stress of this disease.
  • Avoid tobacco smoking.

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Pulmonary Fibrosis