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

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

11. 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

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

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

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

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

16. 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