Idiopathic Pulmonary Fibrosis:Diagnosis and Understanding

John A. Belperio, M.D. 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 rare35 to 44 years old

3 persons per 100,000 population

> 75 years old175 persons per 100,000 population

Clinical Presentation

Insidious onset of symptoms:

SOB

DOE

Cough

Decreased activity

Slowing down

Physical ExamAbnormal 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