Idiopathic Pulmonary Fibrosis

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

• The components of the alveolar wall include:

1. Epithelial cells2. Endothelial cells3. Cellular and

noncellular components of the interstitium

4. The capillary network Red Blood CellRed Blood CellRed Blood CellRed Blood Cell

AlveolusAlveolusAlveolusAlveolus OO22OO22 COCO22COCO22

Pathophysiology

• Reduced lung compliance increase work of breathing (WOB)

• V/Q mismatching• Impaired diffusion

of oxygen to alveolar capillary

• Hypoxemia • Respiratory failure

OO22OO22

OO22OO22

Wasted perfusionWasted perfusionWasted perfusionWasted perfusion

Inadequate OInadequate O22

Inadequate OInadequate O22

Clinical Manifestation

• Insidious or acute onset dyspnea on exertion (DOE) progress to resting dyspnea

• Repetitive nonproductive cough• Fatigue• Loss of appetite, weight loss• Tachypnea

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

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.

Clinical Manifestation

• Bibasilar end-inspiratory rales, and decreased breath sounds

Click here to Click here to listen to an IPF listen to an IPF patient’s breath patient’s breath

soundsound

Clinical Manifestation

• Cyanosis later• Digital clubbing• Cor-polmonale or

respiratory failure

Treatment

• Corticosteroids • Cytoxic drugs e.g. cyclophosphamide

and azathioprine• Combine 1&2 treatment is preferable• Supportive measures e.g. smoking

cessation, oxygenation and ventilation, good nutrition, and aggressive treatment of infection