Updated: 4/28/2021

Emphysema

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Introduction
  • A condition characterized by dilation of air spaces (with ↓ elasticity and ↑ compliance)
    • due to alveolar wall destruction
      • normally, elastin in the wall functions to keep alveoli open
    • capillaries are destroyed along with the alveoli
  • Causes
    • smoking
      • most common cause
    • α1-antitrypsin (AAT) deficiency
      • AD disorder (codominant)
        • MM phenotype is normal
        • ZZ phenotype results in disease
      • AAT normally inhibits the action of elastase
      • without AAT elastase is unchecked and destroys the elastic tissue of the alveoli  
      • as a result emphysema develops at early age
        • may also develop cirrhosis due to the inability to release an abnormal form of AAT from the liver resulting in hepatotoxicity 
Classification
  • Centriacinar  
    • dilated respiratory bronchiole
    • most common presentation of emphysema due to smoking
      • result of inhaled tobacco toxins arriving first in the respiratory bronchioles before traveling to the alveolus
    • most commonly in the upper lobes
      • result of upper lobes receiving exposure to smoke
  • Panacinar
    • dilated alveoli
    • most common presentation of AAT deficiency
      • also due to a functional AAT deficiency as a result of smoking
        • oxidants and inflammatory reaction of smoke can destroy AAT
        • smoking exacerbates effects of genetic AAT deficiency 
    • most commonly in the lower lobes
      • result of lower lobes recieving ↑ perfusion allowing more immune cells to traffic into the alveoli
  • Paraseptal 
    • most commonly involves young, otherwise healthy males
    • does not obstruct the airway
      • associated with bullae
      • found near the pleura
      • increased risk for spontaneous pneumothorax
Presentation
  • Symptoms
    • dyspnea
    • classic pursed-lip breathing
      • results in increased airway pressure and prevents airway collapse during exhalation
  • Physical exam
    • decreased breath sounds on auscultation
    • increased anterior-posterior diameter (barrel chest)
    • hyperresonant to percussion
    • "pink puffer" (end-stage)
Imaging
  • Chest radiograph
    • increased AP diameter with flattened diaphragms 
    • hyperinflated lungs
    • loss of lung markings
    • elongated heart
Evaluation
  • Labs
    • ABG during exacerbation shows hypoxemia and acute respiratory acidosis  
    • AAT shows no α-globin peak on electrophoresis
  • Pulmonary function tests 
    • decreased FEV1 sec / FVC
    • increased TLC and RV  
    • decreased diffusion capacity from destruction of capillaries
Treatment
  • Conservative 
    • smoking cessation
    • ambulatory O2 
  • Pharmacologic
    • bronchodilators 
      • for symptom improvement
    • inhaled steroids
      • reduce exacerbations
    • oral/IV steroids and antibiotics
      • for acute exacerbations

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Questions (10)
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(M1.PL.13.85) A 28-year-old patient presents to the hospital complaining of progressively worsening dyspnea and a dry cough. Radiographic imaging is shown below. Pulmonary function testing (PFT's) reveals a decreased FEV1 and FEV1/FVC, but an increase TLC. The patient states that he does not smoke. Which of the following conditions is most consistent with the patients symptoms?

QID: 100888
FIGURES:
1

Chronic bronchitis

6%

(5/86)

2

Alpha1-antitrypsin deficiency

70%

(60/86)

3

Pneumothorax

1%

(1/86)

4

Asthma

17%

(15/86)

5

Hypersensitivity pneumonitis

6%

(5/86)

M 2 E

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(M1.PL.13.81) A patient with a1-antitrypsin deficiency is warned by his physician that his increasing dyspnea may be worsened by his continued cigarette smoking. Which of the following factors, released by both neutrophils and alveolar macrophages, is responsible for the patient's condition?

QID: 100884
1

Major Basic Protein

8%

(6/80)

2

Antibodies against alpha-3 segment of collagen IV (COL4A3)

6%

(5/80)

3

Mucus

5%

(4/80)

4

Surfactant

0%

(0/80)

5

Elastase

74%

(59/80)

M 1 E

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(M1.PL.13.137) A 65-year-old male presented to his primary care physician with exertional dyspnea. The patient had a 30-year history of smoking one pack of cigarettes per day. Physical examination reveals a barrel-chested appearance, and it is noted that the patient breathes through pursed lips. Spirometry shows decreased FEV1, FVC, and FEV1/FVC. This patient’s upper lobes are most likely to demonstrate which of the following?

QID: 100940
1

Panacinar emphysema

19%

(18/95)

2

Centriacinar emphysema

75%

(71/95)

3

Calcified nodule

2%

(2/95)

4

Hypersensitivity pneumonitis

2%

(2/95)

5

Uncalcified nodule

1%

(1/95)

M 2 E

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(M1.PL.13.45) A 50-year-old Caucasian male presents to the Emergency Department complaining of shortness of breath and unintentional weight loss over the past several months. On physical examination, the patient appears quite thin and breathes through pursed lips. Breath sounds are decreased in all lung fields. The patient's chest x-ray is provided in Figure A. Which of the following findings is expected on spirometry?

QID: 100848
FIGURES:
1

Increased FEV1

1%

(1/74)

2

Decreased FEV1/FVC

93%

(69/74)

3

Decreased TLC

4%

(3/74)

4

Normal FEV1 but increased FVC

1%

(1/74)

5

Normal lung values

0%

(0/74)

M 2 E

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(M1.PL.13.87) A 60-year-old male presents to your office complaining of dyspnea on exertion. He reports smoking two packs of cigarettes per day for the past 25 years. A lung CT is shown in Figure A. Which of the following is LEAST likely to be involved in the pathogenesis of this patient's disease?

QID: 100890
FIGURES:
1

Immunoglobulin A

68%

(47/69)

2

Proteinase 3

9%

(6/69)

3

Neutrophil elastase

3%

(2/69)

4

Proteases released by macrophages

4%

(3/69)

5

Matrix metalloproteinases

12%

(8/69)

M 1 E

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