Respiratory System Pathology - COPD (Emphysema)
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| The major diffuse obstructive disorders are: | 1) Emphysema
2) Chronic Bronchitis
3) Bronchiectasis
4) Asthma
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| Expiratory obstruction may results from either: | 1) Anatomic narrowing, like asthma
2) Loss of elastic recoil, like emphysema
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| There may be overlapping between which obstructive disorders? | Emphysema, bronchitis, and asthma
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| How does one differentiate between emphysema and chronic bronchitis? | Chronic bronchitis is defined on the basis of clinical features
Emphysema is morphologic
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| Which part of the lung does chronic bronchitis involve? | Large airways
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| Which part of the lung does emphysema involve? | The acinus (Bronchioles, alveoli)
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| Can emphysema and chronic bronchitis exist separately? | Yes, but they usually coexist
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| What is the major cause of chronic bronchitis and emphysema? | Heavy tobacco exposure
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| What category of disease do emphysema and chronic bronchitis fall under? | COPD (Chronic obstructive pulmonary disease)
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| What is Emphysema? | Abnormal permanent enlargement of the air distal to the terminal bronchioles accompanied by destruction of their walls without fibrosis, with destruction of the alveolar septa
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| What is the acinus? | The structure distal to terminal bronchioles
Cluster of three to five acini is called a lobule
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| What are the major types of emphysema? | 1) Centracinar
2) Panacinar
3) Distal acinar
4) Irregular
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| What is the centriacinar emphysema? | The central or proximal parts of the acini, formed by respiratory bronchioles are affected, while distal alveoli are spared
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| How can you characterize this disease? | Both emphysematous and normal air spaces exist within the same acinus and lobule
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| Which lesions are more commonly/severly affected in this type of emphysema? | The upper lobes
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| What is the pathogenesis of this disease? | *Exposure to tobacco smoke and pollutants induces ongoing inflammation (neutrophil, macrophage, and lymphocyte accumulation)
*Neutrophils release elastases, cytokines, and oxidants causing epithelial injury and proteolysis of the ECM
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| What is panacinar emphysema? | Emphysema in which the acini are uniformly enlarged from the respiratory bronchioles to the alveoli
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| Which lung zones are more likely to be affected? | The lower lung zones
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| What is the main cause of paracinar emphysema? | a1 antitrypsin deficiency
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| What is distal acinar emphysema also known as? | Paraseptal emphysema
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| What part of the lung does this emphysema involve? | The distal part of the acinus (proximal part is normal)
More striking adjacent to the pleura, along the connective tissue septa
Adjacent to areas of fibrosis
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| What is the characteristic finding of distal acinar emphysema? | Multiple, contiguous, enlarged air spaces
Ranging from less than 0.5 mm to more than 2 cm with bullae
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| What is the most common cause of distal acinar emphysema? | Pneumothorax in young adults
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| What is irregular emphysema? | *The acinus is irregularly involved
*Almost invariably associated with scarring
*Clinically asymptomatic
*may be the most common form of emphysema.
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| The cycle of inflammation and ECM proteolysis continues unless: | checked by antielastases
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| Example of antielastases: | A1 antitrypsin
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| What percentage of patients with congenital a1 antitrypsin deficiency develop panacinar emphysema? | 80%
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| Which genetic polymorphisms control the response to injury after smoking? | 1) TGFB influences susceptibility to COPD by regulating mesenchymal cell response to injury (signalling is reduced, inadequate elastin repair)
2) MMP-9 and MMP-12
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| Morphology of lungs in panacinar emphysema: | Pale, voluminous lungs that obscure the heart
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| Morphology of lungs in centriacinar emphysema: | Lungs are less voluminous, and the upper two thirds are more severely affected
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| Histologic examination shows: | 1) Destruction of alveolar wall without fibrosis
2) Enlarged air spaces
3) Number of alveolar capillaries is diminished
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| Clinical features of emphysema: | 1) Dysnpnea
2) Weight loss - may suggest malignancy
3) Barrel chested patient
4) Prolonged expiration
5) Hunched over position
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| Blood gas values are : | Normal
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| Prominent dyspnea and adequate oxygenation of hemoglobin leads to these patients being called: | Pink puffer
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| When the emphysemic patient also has chronic bronchitis: | Dyspnea usually is less prominent, with diminished respiratory drive, so the patient retains carbon dioxide, becomes hypoxic, and often is cyanotic
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| These patients are called: | Blue bloaters
*They tend to be obese for unknown reasons
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| These patients seek medical help after the onset of: | 1) Cor pulmonale
2) Associated edema
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| What is cor pulmonale? | Right sided heart failure due to abnormalities in the lung
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| In all cases, what condition develops? | Secondary pulmonary hypertension
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| Death from emphysema is due to: | 1) Pulmonary failure with respiratory acidosis, hypoxia, and coma
2) Right sided heart failure (cor pulmonale)
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| Conditions related to emphysema are: | 1) Compensatory Emphysema
2) Obstructive overinflation
3) Bullous emphysema
4) Mediastinal (interstitial) emphysema
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| Why are these conditions more properly referred to as overinflation, rather than emphysema? | They only resemble emphysema superficially
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| What is compensatory emphysema? | The compensatory dilation of alveoli in response to loss of lung substance
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| When can this occur? | In residual lung parenchyma, after surgical removal of a diseased lung or lobe
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| What is obstructive overinflation? | The condition in which the lung expands because air is trapped within it
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| What is a common cause of obstructive overinflation? | Subtotal obstruction by a tumor or foreign object
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| When can it become life threatening? | If the affected portion extends sufficiently to compress the remaining normal lung
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| What is bullous emphysema? | Any form of emphysema that produces large subpleural blebs or bullae
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| What are bullae? | Spaces greater than 1 cm in diameter in the
distended state
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| These blebs represent: | Localized accentuations of any form of emphysema
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| Most of the time, the blebs are where? | Subpleural
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| If these blebs rupture, it will leads to: | Pneumothorax
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| What is Mediastinal (interstitial) emphysema? | Condition resulting when air enters the connective tissue stroma of the lung, mediastinum, and subcutaneous tissue
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| What are the causes of mediastinal emphysema? | 1) Sudden increase in intra alveolar pressure (violent coughing or vomiting/ children with whooping cough)
2) Patients on respirators who have partial bronchiolar obstruction
3) Patients suffering a perforating injury (fractured rib)
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| What effect does a fracture rib have? | Goes to the neck
The head, neck, and chest, swell up and there is crackling crepitation all over the chest (like bubble wrap)
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| If the site of entry is sealed: | The air is resorbed spontaneously
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