FLASHPATH

Hazem Ali
EMPHYSEMA
Hazem Ali
 CLINICAL

Emphysema is one of the obstructive lung diseases

that include:
Chronic bronchitis

Bronchiectasis

Small-airway disease bronchiolitis

Asthma
 CLINICAL
Obstructive airway Restrictive airway
disease disease
General features Increase in resistance to Reduced expansion of
airflow due to obstruction lung parenchyma
at any level
Total lung capacity Increased Reduced
(TLC)
Forced Expiratory Reduced Normal
Volume in one second
(FEV1)
CLINICAL
Emphysema and chronic bronchitis are often clinically
grouped together and referred to as chronic obstructive
pulmonary disease (COPD)

COPD is the 3rd leading cause of global death

Usually due to cigarette smoking and air pollution

Usually present in adults (> 45 years old) and more common

in males

Main symptom are dyspnea and cough

CLINICAL
Predominant Chronic Predominant
Bronchitis Emphysema
Age 40 45 years old 50 75 years old
Appearance Blue Bloater Pink Buffer
Dyspnea Mild, Late Early, Severe
Cough Early Late
Copious sputum Scanty sputum
Infections Common Occasional
Respiratory Repeated Terminal
Insufficiency
Cor pulmonale Common Rare, Terminal
Radiology Prominent vessels Hyperinflation
Large heart Small heart
Airway Resistance Increased Normal or slightly
CLINICAL
Emphysema is:
Permanent enlargement of air spaces
Distal to the terminal bronchioles (= Acini)
Due to destruction of the alveolar septa
Without fibrosis

Three main types:

Centriacinar Panacinar Paraseptal
% 97% 1% 2%
Affected Proximal parts of acini Entire acini Distal parts of acini
segment Respiratory bronchioles = RB + alveolar duct + near septa/pleura
RB alveoli
Lobe Mainly upper lobes Mainly lower lobes Mainly sub-pleura
Cause Smoking 1-antitrypsin Fibrosis, atelectasis
CLINICAL
Centriacinar emphysema can be seen also in coal
workers pneumoconiosis

Panacinar emphysema can be seen also in talc IV

drug abuse and in Ritalin use
CLINICAL
Lines of treatment:
Smoking cessation
Medications (Bronchodilators, Steroids)
Oxygen therapy and Ventilatory support
Surgical (Lung volume reduction surgery, Lung transplant)
Replacement therapy (for alpha-1-antitrypsin deficiency)

Main causes of death:

Coronary artery disease
Respiratory failure
Right-sided heart failure
Massive lung collapse secondary to pneumothorax
 PATHOGENESIS
Inflammatory mediators
Smoking stimulate inflammation & inflammatory mediators
Protease / antiprotease imbalance ( = loss of elastin)
Smoking increases elastase protease
released from neutrophils
Smoking decrease of protective antiproteases
Alpha 1 antitrypsin deficiency deficiency of antiproteases
Oxidative stress
Oxidants are released by smoke substance, damaged tissue, and
inflammatory cells more tissue damage
Genetic predisposition
Some people are genetically more susceptible to these factors than others
GROSS
Increased weight and size of lungs
Overlap hide the heart in situ

Outer surface
Blebs (< 1 cm) or bullae (> 1 cm)
may be seen

Cut section
Large, cystic alveoli
Anthracosis
especially in centriacinar type
GROSS
Centriacinar type:
Both emphysematous (E) and normal airspaces
exist within the same acinus and lobule

Panacinar type:
All airspaces within acini are evenly enlarged

Paraseptal type:
Subpleural airspaces is mainly affected
MICROSCOPY
MICROSCOPY
Histological landmarks of lung acinus:
Bronchovascular bundle terminal bronchiole and arteriole
In the center of acinus
Connective tissue septa "secondary lobule of Millar"
At the periphery of acinus
Often ambiguous in less inflamed lung
Helpful to determine emphysema types
Upper pic Centriacinar
Lower pic Panacinar
MICROSCOPY
Emphysema is characterized by:
Abnormal enlargement of airspaces

Floating alveolar walls fragments within enlarged

airspace

No or little fibrosis

Any degrees of chronic inflammation

The pores of Kohn are so large that

septa appear to be floating or
protrude blindly into alveolar
spaces with a clubshaped end
MICROSCOPY
Other Smoking-related conditions:
Anthracosis
Desquamative interstitial pneumonia
Small airway disease
Respiratory bronchiolitis
Respiratory bronchiolitis-associated interstitial lung disease
Large airway disease
Chronic bronchitis
Pulmonary eosinophilic granuloma
Langerhans cell histiocytosis
Interstitial fibrosis

Pulmonary hypertension
DIFFERENTIAL DIAGNOSIS
Other types of emphysema:
Combined pulmonary fibrosis and emphysema (CPFE)
Coexistence of interstitial fibrosis and emphysema of unknown causes
Poor prognosis in comparison to pure emphysema or pure lung fibrosis

Interstitial emphysema
Air gains access to the pulmonary interstitium to cause air leak and
pneumothorax
Common in premature infants

Bullous emphysema
Formation of multiple bullae > 1 cm with thin wall to cause
pneumothorax
DIFFERENTIAL DIAGNOSIS
Other types of emphysema:
Senile emphysema
Due to age related alteration of acini

Irregular emphysema
Occurs in relation to scars

Congenital lobar emphysema

Hyperinflation of one or more lobes due to malformation of bronchioles
Causes respiratory distress
 DIFFERENTIAL DIAGNOSIS
Other obstructive lung diseases:
Small-
airway
Chronic Bronchiecta
Asthma disease Emphysema
bronchitis sis
bronchiolitis

Site Bronchus Bronchioles Alveoli
Major Mucous Airway Smooth Inflammator Airspace
patholog gland dilation & muscle y scarring & enlargemen
y hyperplasia scarring hyperplasia obliteration t
Excess Excess Wall
mucus mucus destruction
Inflammation Inflammation No fibrosis
(eosinophils)
DIFFERENTIAL DIAGNOSIS
Other congenital / cystic lung diseases:
Congenital:
Bronchogenic cysts
Congenital pulmonary cysts
Congenital pulmonary airway malformation
Congenital lobar emphysema No destruction of alveoli
Pulmonary sequestration
Acquired:
Healed abscess
Fibrosis
Honeycombing
Mixed:
Cystic fibrosis
DIFFERENTIAL DIAGNOSIS
Autolysis and inadequate fixation
Similar gross and microscopic appearance can be seen due to
inadequate inflation or fixation of the specimen with formalin
Clinical history
Favor emphysema
Other smoking-associated conditions
DIFFERENTIAL DIAGNOSIS
Lymphangioleiomyomatosis Vs Emphysema?
Women of reproductive age
Multiple cysts and blebs of the lung
Mural thickening around some cystic spaces
Chylous pleural effusion
Interstitial proliferation of smooth muscle-like cells
around airways and blood vessels
Tumor cells are positive for HMB45
Other Tuberous Sclerosis-Associated lesions (e.g.
PEComas)
DIFFERENTIAL DIAGNOSIS
Placental transmogrification of the lung
Very rare disease
Mainly seen in middle-aged males
Historically considered a microscopic variant
of emphysema
Solitary giant bullous
With/without solid nodules
Papillary/villous structures resembling chorionic villi within
cystic space
Villi cores contain fat cells and primitive mesenchymal clear cells
Can be associated with pulmonary fibrochondromatous
hamartomas
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Hazem Ali