Idiopathic Pulmonary Fibrosis: Diagnosis and Understanding John A. Belperio, M.D. UCLA School of Medicine, Division of Pulmonary and Critical Care Medicine

INTRODUCTION What is IPF Specific form of fibrosing interstitial pneumonitis referred to as usual interstitial pneumonia

Epidemiology Prevalence in the general population 3 to 175 person per 100,000 population < 50 years old rare 35 to 44 years old 3 persons per 100,000 population > 75 years old 175 persons per 100,000 population

Clinical Presentation Insidious onset of symptoms: SOB DOE Cough Decreased activity Slowing down

Physical Exam Abnormal Breath sound Digital Clubbing Look for evidence of connective tissue disease

Diagnosis Pulmonary Function Testing Chest X - Ray CAT SCAN Bronchoscopy Lung Biopsy

CONCLUSIONS Insidious onset of symptoms High index of suspicion Appropriate test Appropriate procedure to make the diagnosis Appropriate treatment New treatments

Genetics of Pulmonary fibrosis Exclude collagen vascular disease Exclude occupational disease Exclude medications associated with pulmonary fibrosis Clustering of pulmonary fibrosis seen in families IPF seen in separately raised monozygotic twins IPF seen in more than two generations in several studies, and in several father son pairs