5 - Bullous Emphysema and PTX

Question 1

% of adults with bullous emphysema with severe clinical disease

Answer 1

10%

Question 2

% of cigarette smokers that have significant chronic airflow obstruction assocaited with chronic bronchitis

Answer 2

10%

Question 3

Pathogenesis of bullous emphysema

Answer 3

Enzymatic tissue destruction (protease pathogenesis hypothesis)

Question 4

What is protease pathogenesis hypothesis for bullous emphysema

Answer 4

Destruction of pulmonary interstitium due to excess protolytic enzymes (elastase) relative to the availability of proteolytic inhibitors (alpha 1 anti-trypsin)

Question 5

Alpha 1 anti-trypsin a.k.a.

Answer 5

Alpha-1 protease inhibitor

Question 6

MOA of cigarette smoke on alpha 1 anti-trypsin

Answer 6

oxidate inactivation of alpha-1 anti-trypsin by oxidants in smoke or secondarilyby active oxygen species liberated by pulmonary phagocytes

Question 7

Characteristics of alveolar macrophages in smokers

Answer 7

increased amoutns of oxygen radicals and peroxide

Question 8

Characteristics of alpha-1 anti-trypsin

Answer 8

Plasma protease inhibitor
Synthesized in liver
Primary site of action: alveolar structure of lung
Inhibits PMS leukocyte elastase
Protects elastic fibers from hydrolysis

Question 9

Alpha-1 antitrypsin deficiency results

Answer 9

20-30x increased risk of pulmonary disease

Question 10

% of emphysema population with alpha-1 anti-trypsin def

Answer 10

1-2%

Question 11

Inheritance of apha-1 anti-trypsin

Answer 11

Autosomal recessive

Question 12

4 Anatomic Classifications of Emphysema

Answer 12

Proximal acinar (centrilobular)
Panaciner (panlobular)
Distal acinar (paraseptal)
Irregular

Question 13

Anatomic emphysema classification associated with upper lung zones

Answer 13

Proximal acinar (centrilobular)

Question 14

Anatomic emphysema classification associated with uneven distribution

Answer 14

Proximal acinar (centrilobular)

Question 15

Anatomic emphysema classification associated with cigarette smoking and inflammation of distal airways

Answer 15

Proximal acinar (centrilobular)

Question 16

Anatomic emphysema classification associated with entire acinus involvment uniformily

Answer 16

Panacinar

Question 17

Anatomic emphysema classification associated with alpha-1 anti-trypsin def

Answer 17

Panacinar

Question 18

Anatomic emphysema classification associated with lower lung zone distribution

Answer 18

Panacinar

Question 19

Anatomic emphysema classification associated with distal acinus, ducts and alveolar sacs

Answer 19

Distal acinar (paraseptal)

Question 20

Anatomic emphysema classification associated with subpleral location (PTX and bullous dis)

Answer 20

Distal acinar

Question 21

Anatomic emphysema classification associated with scarring and fibrosis (always)

Answer 21

Irregular

Question 22

3 Clinical Classificatons of Emphysema

Answer 22

Compensatory emphysema
Diffuse Obstructive
Bullous Disease

Question 23

Description of Compensatory Emphysema

Answer 23

Not true emphysema (no acinar destruction)
Infantile lobar emphysema (localized emphysema due to airway obstruction [tumor, infection, foreign body aspiration])
Compensatory changes after lobectomy

Question 24

Classification of emphysema that is a major component of COPD

Answer 24

Diffuse objects

Question 25

Type A Diffuse Obstructive Emphysema

Answer 25

"Pink Puffer"
Dry cough and dyspnea
Barrell chest
Pan-acinar distribution
Good prognosis
Major complication is PTX

Question 26

Type B Diffuse Obstructive Emphysema

Answer 26

“Wet Emphysema”
Severe cought and sputum production
CXR with moderate hyperinflation, fibrosis in lower lobes
Centrilobular changes
Poor prognosis
Major complicatin is retention of secretions and respiratory failure.