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EMPHYSEMA



[COPD - CHRONIC OBSTRUCTIVE
    PULMONARY DISEASES]
      Dr.CSBR.Prasad, M.D.
Clinical presentations


• Dyspnoea
• Recurrent respiratory infections
• Right heart failure
Main pulmonary change



Increased air space in the lungs
Emphysema

Definition: Abnormal permanent
 enlargement of the airspaces distal to the
 terminal bronchiole, accompanied by
 destruction of their walls and without
 obvious fibrosis.



Spaces in parenchyma   > 1mm   = Abnormal
Emphysema


 Emphysema causes dilation of air
spaces by destruction of alveolar wall,
 leading to collapse of alveoli during
              expiration
Emphysema & Overinflation
• Emphysema: Increased air space with
  destruction
• Overinflation: Increased air space without
  destruction
Posteroanterior (PA) and lateral chest radiograph in a patient
with severe chronic obstructive pulmonary disease (COPD).
Hyperinflation, depressed diaphragms, increased retrosternal
space, and hypovascularity of lung parenchyma is demonstrated.
A lung with
  emphysema shows
      increased
anteroposterior (AP)
 diameter, increased
retrosternal airspace,
    and flattened
diaphragms on lateral
  chest radiograph.
Severe bullous
   disease
 observed on
 CT scan in a
 patient with
   COPD
Classification: Types of emphysema

1. Centriacinar
2. Panacinar
3. Paraseptal [Distal acinar]
4. Mixed & unclassified [Irregular]
RB1


            Normal
            Acinus




  Alveoli
A
     RB       Centrilobular
TB



               Panlobular



               Paraseptal


                Irregular
Centriacinar :
    [ centrilobular, Proximal acinar ]

•   Dilatation of Respiratory Bronchiole
•   Upper lobes - severely involved
•   Can coexist with chronic bronchitis
•   Invariably occurs in smokers
•   Coal mine workers [carbon, dust]
CENTRIACINAR




FIGURE 15-7 A, Centriacinar emphysema. Central areas
show marked emphysematous damage (E), surrounded by
relatively spared alveolar spaces. B, Panacinar emphysema
involving the entire pulmonary lobule.
CENTRIACINAR
Panacinar Emphysema:

• Whole of Acinus uniformly affected
• Lower lobes severely involved
• Association:
     … A1AT deficiency
     … Cigarette smokers
PANACINAR
Paraseptal (Distal Acinar)

• Localized along pleura - peripheral part
  of the acinus
• Predisposes to spontaneous peumothorax
• Adjacent to foci of fibrosis
• Least common
Mixed – IRREGULAR EMPHYSEMA:

 •   MOST COMMON
 •   LEAST SIGNIFICANT
 •   COMMON AROUND SCAR TISSUE
 •   COMBINATION OF TYPES
Mixed [CENTRIACINAR + PARASEPTAL]
Microscopy of emphysema
Emphysema - Microscopy
Pathogenesis

• Protease and antiprotease theory
    AAT, A1MG
• Oxidant-antioxidant imbalance
    SOD, Glutathione
Alpha-1 Antitrypsin deficiency

•   52 kD serum glycoprotein
•   Synthesis: liver, macrophage
•   Inhibits - Trypsin, Thrombin, Plasmin, Elastase
•   Gene: chromosome 14 [75 alleles]
        * Normal allele -- MM (90%)
        * Deficiency      -- ZZ
Pathophysiology
Pathophysiology

• Expiratory narrowing of bronchioles >
   Air flow obstruction > underventilation

• Mismatch between ventilation & perfusion

• Can lead to chronic cor pulmonale
Etiology
• Cigarette smoking [20 - 40% smokers]
• Air pollution
• Alpha-1-antitrypsin deficiency
• Inherited diseases ( Rare )
   * Cutis laxa
   * Marfan’s syndrome
   * Menke’s syndrome
Cutis Laxa
Marfan’s syndrome
Elher Danlos syndrome
Menke’s syndrome
Other types of emphysema
•   Compensatory emphysema
•   Senile emphysema
•   Obstructive overinflation
•   Bullous emphysema
•   Interstitial emphysema:
             … Trauma to chest wall
             … Severe cough
Bullae
• Def: Subpleural, air-filled cystic areas
• Size: >1cm
• Location: Anterior margin & Apices

• Complication: Rupture > Pneumothorax
Emphysematous
   Bullae
Fig-1 Bullous emphysema with large subpleural bullae (upper left)
Fig-2 Chronic obstructive pulmonary disease (COPD). Gross pathology of a patient with
Irregular Emphysema with Bullae
Atelectasis of right lung with shift in mediastinum
Clinical picture
• Dysponea
• Cough with or without expectoration
• Wheezing
• Loss of weight
• Peptic ulceration
• Hypercapnia > changes in central
  nervous system
• Barrel chest
Barrel chest
Obstructive Pulmonary diseases
• Disorders Associated with Airflow
  Obstruction
• Chronic bronchitis, Emphysema, Asthma,
  Bronchiectasis & Bronchiolitis come under
  this category
Venn diagram of chronic obstructive pulmonary disease (COPD).
  Chronic obstructive lung disease is a disorder in which subsets of
    patients may have dominant features of chronic bronchitis,
emphysema, or asthma. The result is irreversible airflow obstruction.
COPD
• COPD: Comprises Emphysema and chronic
  bronchitis
• Many patients have overlapping features of
  damage at both the acinar level (emphysema) and
  bronchial level (bronchitis)
• Common extrinsic trigger— cigarette smoking —
  is implicated in both the diseases
Figure 15-9 Schematic representation of evolution of
   chronic bronchitis (left) and emphysema (right).
Natural history of COPD
• Pathological process (for years)
   > clinical symptoms
• Survival is variable
• Respiratory failure > terminal phase of disease
• 2/3 dead < 2 years
• DEATH: - Respiratory acidosis and coma
           - Chronic cor pulmonale
           - Spontaneous pneumothorax
“Pink Puffer”

                Emphysema
                Thin
                Dysponea
                < Vital capacity
                > Lung volume
“Blue Bloater” – Chronic bronchitis
“Pink Puffers & Blue Bloaters”
Table 15-4 -- Emphysema and Chronic Bronchitis


                              Predominant Bronchitis       Predominant Emphysema

Age (yr)                    40–45                          50–75
Dyspnea                     Mild; late                     Severe; early
Cough                       Early; copious sputum          Late; scanty sputum
Infections                  Common                         Occasional
Respiratory insufficiency   Repeated                       Terminal
Cor pulmonale               Common                         Rare; terminal
Airway resistance           Increased                      Normal or slightly increased
Elastic recoil              Normal                         Low
Chest radiograph            Prominent vessels; large heart Hyperinflation; small heart
Appearance                  Blue bloater                   Pink puffer
E N D

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Emphysema

  • 1. EMPHYSEMA [COPD - CHRONIC OBSTRUCTIVE PULMONARY DISEASES] Dr.CSBR.Prasad, M.D.
  • 2. Clinical presentations • Dyspnoea • Recurrent respiratory infections • Right heart failure
  • 3. Main pulmonary change Increased air space in the lungs
  • 4.
  • 5. Emphysema Definition: Abnormal permanent enlargement of the airspaces distal to the terminal bronchiole, accompanied by destruction of their walls and without obvious fibrosis. Spaces in parenchyma > 1mm = Abnormal
  • 6. Emphysema Emphysema causes dilation of air spaces by destruction of alveolar wall, leading to collapse of alveoli during expiration
  • 7.
  • 8. Emphysema & Overinflation • Emphysema: Increased air space with destruction • Overinflation: Increased air space without destruction
  • 9. Posteroanterior (PA) and lateral chest radiograph in a patient with severe chronic obstructive pulmonary disease (COPD). Hyperinflation, depressed diaphragms, increased retrosternal space, and hypovascularity of lung parenchyma is demonstrated.
  • 10. A lung with emphysema shows increased anteroposterior (AP) diameter, increased retrosternal airspace, and flattened diaphragms on lateral chest radiograph.
  • 11. Severe bullous disease observed on CT scan in a patient with COPD
  • 12. Classification: Types of emphysema 1. Centriacinar 2. Panacinar 3. Paraseptal [Distal acinar] 4. Mixed & unclassified [Irregular]
  • 13. RB1 Normal Acinus Alveoli
  • 14.
  • 15. A RB Centrilobular TB Panlobular Paraseptal Irregular
  • 16. Centriacinar : [ centrilobular, Proximal acinar ] • Dilatation of Respiratory Bronchiole • Upper lobes - severely involved • Can coexist with chronic bronchitis • Invariably occurs in smokers • Coal mine workers [carbon, dust]
  • 17. CENTRIACINAR FIGURE 15-7 A, Centriacinar emphysema. Central areas show marked emphysematous damage (E), surrounded by relatively spared alveolar spaces. B, Panacinar emphysema involving the entire pulmonary lobule.
  • 19. Panacinar Emphysema: • Whole of Acinus uniformly affected • Lower lobes severely involved • Association: … A1AT deficiency … Cigarette smokers
  • 21. Paraseptal (Distal Acinar) • Localized along pleura - peripheral part of the acinus • Predisposes to spontaneous peumothorax • Adjacent to foci of fibrosis • Least common
  • 22. Mixed – IRREGULAR EMPHYSEMA: • MOST COMMON • LEAST SIGNIFICANT • COMMON AROUND SCAR TISSUE • COMBINATION OF TYPES
  • 23. Mixed [CENTRIACINAR + PARASEPTAL]
  • 26. Pathogenesis • Protease and antiprotease theory AAT, A1MG • Oxidant-antioxidant imbalance SOD, Glutathione
  • 27.
  • 28.
  • 29.
  • 30. Alpha-1 Antitrypsin deficiency • 52 kD serum glycoprotein • Synthesis: liver, macrophage • Inhibits - Trypsin, Thrombin, Plasmin, Elastase • Gene: chromosome 14 [75 alleles] * Normal allele -- MM (90%) * Deficiency -- ZZ
  • 32. Pathophysiology • Expiratory narrowing of bronchioles > Air flow obstruction > underventilation • Mismatch between ventilation & perfusion • Can lead to chronic cor pulmonale
  • 33. Etiology • Cigarette smoking [20 - 40% smokers] • Air pollution • Alpha-1-antitrypsin deficiency • Inherited diseases ( Rare ) * Cutis laxa * Marfan’s syndrome * Menke’s syndrome
  • 38. Other types of emphysema • Compensatory emphysema • Senile emphysema • Obstructive overinflation • Bullous emphysema • Interstitial emphysema: … Trauma to chest wall … Severe cough
  • 39. Bullae • Def: Subpleural, air-filled cystic areas • Size: >1cm • Location: Anterior margin & Apices • Complication: Rupture > Pneumothorax
  • 40. Emphysematous Bullae
  • 41. Fig-1 Bullous emphysema with large subpleural bullae (upper left) Fig-2 Chronic obstructive pulmonary disease (COPD). Gross pathology of a patient with
  • 43. Atelectasis of right lung with shift in mediastinum
  • 44. Clinical picture • Dysponea • Cough with or without expectoration • Wheezing • Loss of weight • Peptic ulceration • Hypercapnia > changes in central nervous system • Barrel chest
  • 46.
  • 47. Obstructive Pulmonary diseases • Disorders Associated with Airflow Obstruction • Chronic bronchitis, Emphysema, Asthma, Bronchiectasis & Bronchiolitis come under this category
  • 48. Venn diagram of chronic obstructive pulmonary disease (COPD). Chronic obstructive lung disease is a disorder in which subsets of patients may have dominant features of chronic bronchitis, emphysema, or asthma. The result is irreversible airflow obstruction.
  • 49. COPD • COPD: Comprises Emphysema and chronic bronchitis • Many patients have overlapping features of damage at both the acinar level (emphysema) and bronchial level (bronchitis) • Common extrinsic trigger— cigarette smoking — is implicated in both the diseases
  • 50. Figure 15-9 Schematic representation of evolution of chronic bronchitis (left) and emphysema (right).
  • 51. Natural history of COPD • Pathological process (for years) > clinical symptoms • Survival is variable • Respiratory failure > terminal phase of disease • 2/3 dead < 2 years • DEATH: - Respiratory acidosis and coma - Chronic cor pulmonale - Spontaneous pneumothorax
  • 52.
  • 53. “Pink Puffer” Emphysema Thin Dysponea < Vital capacity > Lung volume
  • 54. “Blue Bloater” – Chronic bronchitis
  • 55. “Pink Puffers & Blue Bloaters”
  • 56. Table 15-4 -- Emphysema and Chronic Bronchitis Predominant Bronchitis Predominant Emphysema Age (yr) 40–45 50–75 Dyspnea Mild; late Severe; early Cough Early; copious sputum Late; scanty sputum Infections Common Occasional Respiratory insufficiency Repeated Terminal Cor pulmonale Common Rare; terminal Airway resistance Increased Normal or slightly increased Elastic recoil Normal Low Chest radiograph Prominent vessels; large heart Hyperinflation; small heart Appearance Blue bloater Pink puffer
  • 57. E N D