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