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RS Pathology

Respiratory System Pathology - COPD (Emphysema)

The major diffuse obstructive disorders are: 1) Emphysema 2) Chronic Bronchitis 3) Bronchiectasis 4) Asthma
Expiratory obstruction may results from either: 1) Anatomic narrowing, like asthma 2) Loss of elastic recoil, like emphysema
There may be overlapping between which obstructive disorders? Emphysema, bronchitis, and asthma
How does one differentiate between emphysema and chronic bronchitis? Chronic bronchitis is defined on the basis of clinical features Emphysema is morphologic
Which part of the lung does chronic bronchitis involve? Large airways
Which part of the lung does emphysema involve? The acinus (Bronchioles, alveoli)
Can emphysema and chronic bronchitis exist separately? Yes, but they usually coexist
What is the major cause of chronic bronchitis and emphysema? Heavy tobacco exposure
What category of disease do emphysema and chronic bronchitis fall under? COPD (Chronic obstructive pulmonary disease)
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
What is the acinus? The structure distal to terminal bronchioles Cluster of three to five acini is called a lobule
What are the major types of emphysema? 1) Centracinar 2) Panacinar 3) Distal acinar 4) Irregular
What is the centriacinar emphysema? The central or proximal parts of the acini, formed by respiratory bronchioles are affected, while distal alveoli are spared
How can you characterize this disease? Both emphysematous and normal air spaces exist within the same acinus and lobule
Which lesions are more commonly/severly affected in this type of emphysema? The upper lobes
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
What is panacinar emphysema? Emphysema in which the acini are uniformly enlarged from the respiratory bronchioles to the alveoli
Which lung zones are more likely to be affected? The lower lung zones
What is the main cause of paracinar emphysema? a1 antitrypsin deficiency
What is distal acinar emphysema also known as? Paraseptal emphysema
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
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
What is the most common cause of distal acinar emphysema? Pneumothorax in young adults
What is irregular emphysema? *The acinus is irregularly involved *Almost invariably associated with scarring *Clinically asymptomatic *may be the most common form of emphysema.
The cycle of inflammation and ECM proteolysis continues unless: checked by antielastases
Example of antielastases: A1 antitrypsin
What percentage of patients with congenital a1 antitrypsin deficiency develop panacinar emphysema? 80%
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
Morphology of lungs in panacinar emphysema: Pale, voluminous lungs that obscure the heart
Morphology of lungs in centriacinar emphysema: Lungs are less voluminous, and the upper two thirds are more severely affected
Histologic examination shows: 1) Destruction of alveolar wall without fibrosis 2) Enlarged air spaces 3) Number of alveolar capillaries is diminished
Clinical features of emphysema: 1) Dysnpnea 2) Weight loss - may suggest malignancy 3) Barrel chested patient 4) Prolonged expiration 5) Hunched over position
Blood gas values are : Normal
Prominent dyspnea and adequate oxygenation of hemoglobin leads to these patients being called: Pink puffer
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
These patients are called: Blue bloaters *They tend to be obese for unknown reasons
These patients seek medical help after the onset of: 1) Cor pulmonale 2) Associated edema
What is cor pulmonale? Right sided heart failure due to abnormalities in the lung
In all cases, what condition develops? Secondary pulmonary hypertension
Death from emphysema is due to: 1) Pulmonary failure with respiratory acidosis, hypoxia, and coma 2) Right sided heart failure (cor pulmonale)
Conditions related to emphysema are: 1) Compensatory Emphysema 2) Obstructive overinflation 3) Bullous emphysema 4) Mediastinal (interstitial) emphysema
Why are these conditions more properly referred to as overinflation, rather than emphysema? They only resemble emphysema superficially
What is compensatory emphysema? The compensatory dilation of alveoli in response to loss of lung substance
When can this occur? In residual lung parenchyma, after surgical removal of a diseased lung or lobe
What is obstructive overinflation? The condition in which the lung expands because air is trapped within it
What is a common cause of obstructive overinflation? Subtotal obstruction by a tumor or foreign object
When can it become life threatening? If the affected portion extends sufficiently to compress the remaining normal lung
What is bullous emphysema? Any form of emphysema that produces large subpleural blebs or bullae
What are bullae? Spaces greater than 1 cm in diameter in the distended state
These blebs represent: Localized accentuations of any form of emphysema
Most of the time, the blebs are where? Subpleural
If these blebs rupture, it will leads to: Pneumothorax
What is Mediastinal (interstitial) emphysema? Condition resulting when air enters the connective tissue stroma of the lung, mediastinum, and subcutaneous tissue
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)
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)
If the site of entry is sealed: The air is resorbed spontaneously
Created by: Ulaisl