Idiopathic Pulmonary Fibrosis

1
Idiopathic Pulmonary Fibrosis

• It is an inflammation process involving all of
  the components of the alveolar wall
• The components of the alveolar wall include

Alveolus
O2
CO2

1. Epithelial cells
2. Endothelial cells
3. Cellular and noncellular components of the
   interstitium
4. The capillary network

Red Blood Cell
2
Pathophysiology
Inadequate O2
O2

• Reduced lung compliance ? increase work of
  breathing (WOB)
• V/Q mismatching
• Impaired diffusion of oxygen to alveolar
  capillary
• Hypoxemia
• Respiratory failure

O2
Wasted perfusion
3
Clinical Manifestation

• Insidious or acute onset dyspnea on exertion
  (DOE) progress to resting dyspnea
• Repetitive nonproductive cough
• Fatigue
• Loss of appetite, weight loss
• Tachypnea

4
Clinical Manifestation

• Diffuse reticulonodular pattern in the involved
  areas on chest x-ray (patient may have a normal
  x-ray)
• Decreased PO2, during exercise, later at rest,
  and normal PCO2

• Normal PO2 80-100, Normal PCO2 35-45

5
Clinical Manifestation

• An abnormal chest x-ray shows scarring and cyst
  formation in both lungs, predominantly in the
  middle and lower areas. These findings are
  typical of idiopathic pulmonary fibrosis. A
  normal chest x-ray is shown on the right for
  comparison the heart (H), lungs (L), vertebrae
  (v), and clavicle (C) can be seen.

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Clinical Manifestation

• Bibasilar end-inspiratory rales, and decreased
  breath sounds

Click here to listen to an IPF patients breath
sound
7
Clinical Manifestation

• Cyanosis later
• Digital clubbing
• Cor-polmonale or respiratory failure

8
Treatment

• Corticosteroids
• Cytoxic drugs e.g. cyclophosphamide and
  azathioprine
• Combine 12 treatment is preferable
• Supportive measures e.g. smoking cessation,
  oxygenation and ventilation, good nutrition, and
  aggressive treatment of infection