Idiopathic Pulmonary Fibrosis: Diagnosis and Understanding

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Idiopathic Pulmonary FibrosisDiagnosis and
Understanding

• John A. Belperio, M.D.

UCLA School of Medicine, Division of Pulmonary
and Critical Care Medicine
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INTRODUCTION

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

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Epidemiology

• Prevalence in the general population

3 to 175 person per 100,000 population
lt 50 years old rare 35 to 44 years old 3 persons
per 100,000 population
gt 75 years old 175 persons per 100,000 population
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Clinical Presentation

• Insidious onset of symptoms

SOB
DOE
Cough
Decreased activity
Slowing down
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Physical Exam

• Abnormal Breath sound

Digital Clubbing
Look for evidence of connective tissue disease
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Diagnosis

• Pulmonary Function Testing

Chest X - Ray
CAT SCAN
Bronchoscopy
Lung Biopsy
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CONCLUSIONS

• Insidious onset of symptoms

High index of suspicion
Appropriate test
Appropriate procedure to make the diagnosis
Appropriate treatment
New treatments
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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