Pulmonary emphysema

SOM-426th group of MNUMS

Students: Altankhuyag.N
Anujin.M
Ariun-Undral.G
Bayanzul.A
Contents
• Definition
• Etiology
• Classification
• Pathophysiology
• Clinical presentation
Definition
• Emphysema is pathologically defined as an abnormal permanent
enlargement of air spaces distal to the terminal bronchioles,
accompanied by the destruction of alveolar walls and without
obvious fibrosis.
• This process leads to reduced gas exchange, changes in airway dynamics that
impair expiratory airflow, and progressive air trapping.

https://emedicine.medscape.com/article/298283-overview
Etiology
• Cigarette smoking [20 - 40% smokers]
• Air pollution
• Alpha-1-antitrypsin deficiency
• Inherited diseases ( Rare )
* Cutis laxa
* Marfan’s syndrome
* Menke’s syndrome
* Ehlers-Danlos syndrome
Classification
• Types of emphysema
• 1. Centriacinar
• 2. Panacinar
• 3. Paraseptal [Distal acinar]
• 4. Mixed & unclassified [Irregular]
Normal and damaged acinus
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]

robbins pathology
Panacinar Emphysema:
• Whole of Acinus uniformly affected
• Lower lobes severely involved
• Association:
• A1AT deficiency
• Cigarette smokers

robbins pathology
Paraseptal (Distal Acinar)
• Localized along pleura - peripheral part of the acinus
• Predisposes to spontaneous peumothorax
• Adjacent to foci of fibrosis
• Least common

robbins pathology
Mixed – IRREGULAR EMPHYSEMA:
• Most commom
• Acinus is irregularly involved
• Associated with scarring and inflammation
• Combination of types

robbins pathology
Pathophysiology
• 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

Color atlas of pathophysiology

Pathophysiology
Pathophysiology - Tobacco

https://www.medicinembbs.org/2011/02/pathogenesis-of-emphysema.html
Color atlas of pathophysiology
Color atlas of pathophysiology
Clinical picture
• Dyspnea
• Cough with or without expectoration
• Wheezing
• Loss of weight
• Peptic ulceration
• Hypercapnia > changes in central nervous system
• Barrel chest
Dyspnea- mechanism
• Hypoxemia may stimulate peripheral chemoreceptors, increasing
ventilator drive from the brainstem.
• Hypercapnia may directly cause ‘air hunger’ but also increased
central ventilatory drive (to blow off carbon dioxide) and corollary
discharge, as discussed above.
• Increased airways resistance and hyperinflation increases the load
that the respiratory muscles must work against, thereby stimulating
muscle receptors.

Mechanisms of clinical sign

Barrel chest
Mechanism: Considered to be due to
over-activity of the scalene and
sternocleidomastoid muscles, which
lift the upper ribs and sternum. With
time, this overuse causes remodeling
of the chest.

Mechanisms of clinical sign