Definition
- Abnormal permanent increase in the size of air spaces beyond the terminal bronchioles
- With destruction of their walls
- Without fibrosis
Pathogenesis
Imbalance between protease & anti-protease activity
- Without fibrosis
Pathogenesis
Imbalance between protease & anti-protease activity
... leads to...
Neutrophils & macrophages = released proteases
Alpha-1-antitrypsin most common protease inhibitor
h/e...
Neutrophils = also release O2 free radicals
h/e...
Neutrophils = also release O2 free radicals
+ oxidants from cigarette smoke
Leads to inhibition of alpha-1-antitrypsin
Leads to inhibition of alpha-1-antitrypsin
Classification
Centriacinar emphysema
- assoc with cig smoking & CB
- increased protease activity
- affects upper zone of lungs
- 2ry to airway inflammation
Panacinar emphysema
- assoc with alpha-1-antitrypsin deficiency
- assoc with cig smoking & CB
- increased protease activity
- affects upper zone of lungs
- 2ry to airway inflammation
Panacinar emphysema
- assoc with alpha-1-antitrypsin deficiency
- decreased anti-protease activity
- affects lower zones of lungs
- homozygotes (PiZZ) = emphysema <40
- heterozygotes = emphysema if they smoke
Pathology
Macro
Cystic alveolar spaces (end-stage emphysema)
Micro
Destruction & dilatation of alveolar walls
Effects
Functional obstruction
- loss of elastic recoil
- premature closure of small airways
- affects lower zones of lungs
- homozygotes (PiZZ) = emphysema <40
- heterozygotes = emphysema if they smoke
Pathology
Macro
Cystic alveolar spaces (end-stage emphysema)
Micro
Destruction & dilatation of alveolar walls
Effects
Functional obstruction
- loss of elastic recoil
- premature closure of small airways
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