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 cells 2.Endothelial cells 3.Cellular and noncellular components of the interstitium 4.The capillary network Red Blood Cell AlveolusAlveolus O2O2O2O2 O2O2O2O2 CO 2

Pathophysiology Reduced lung compliance  increase work of breathing (WOB) V/Q mismatching Impaired diffusion of oxygen to alveolar capillary Hypoxemia Respiratory failure O2O2O2O2 O2O2O2O2 O2O2O2O2 O2O2O2O2 Wasted perfusion Inadequate O 2

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= , 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 listen to an IPF patient’s breath sound

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