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Diseases of Respiratory System

Dr. Faten Ghazal


Prof. of Pathology, Ain Shams University
Lecture 4

Emphysema
Lung Collapse

By the end of this lecture you will able to :


Define emphysema and describe its different types.
types
Explain the pathogenesis of emphysema.
Describe the pathologic features of emphysema,
recognize its clinical picture & list its complications .
Describe the conditions that mimic emphysema.
Define lung collapse & describe its different types.
List the main characteristics of obstruction collapse
Give reasons :1.The centriacinar emphysema
occurring in smokers is more common in the upper lobe ,
while the panacinar emphysema occurring in alpha1
deficient individuals is more common in lower lobe.
lobe
2. Early treatment of lung collapse is
essential.
essential

Pulmonary Emphysema

LUNG DISEASES
To remind you, emphysema is one of the
major diffuse obstructive diseases,
diseases which
include as well chronic bronchitis, bronchial
asthma, & bronchiectasis. In these patients
there is limitation of maximal airflow rates
during forced expiration (increased
resistance to airflow).
Expiratory airflow obstruction may result
from airway narrowing as in asthma or from
loss of elastic recoil which characteristically
occurs in emphysema.

LUNG DISEASES
Diffuse pulmonary diseases can be classified into 2 categories
Obstructive Disease
Restrictive Disease
It is ch.ch. by increased
resistance to airflow due to

It is ch.ch. by reduced expansion


of lung parenchyma with decreased

partial or complete obstruction .

total lung capacity.

The major obstructive diseases


after excluding tumors & inhalation
of foreign body are:

The restrictive diseases occur in 2


conditions:

1. Bronchial Asthma (anatomic airway

1. Acute and chronic interstitial


lung diseases e.g. acute

narrowing)

2. Emphysema (loss of elastic


recoil)

3. Chronic bronchitis (both 2&3 are


called COPD)

4. Bronchiectasis
5. Cystitis fibrosis
6. Bronchiolitis

respiratory distress syndrome and


chronic diseases as
pneumoconiosis,
pneumoconiosis sarcoidosis &
idiopathic pulmonary fibrosis.

2. Extra pulmonary disorders e.g.


obesity, poliomyelitis, pleural
diseases and kyphoscoliosis.

Normal Anatomy

Pulmonary Emphysema

The cut surface of a normally distended lung shows


hexagonal areas of parenchyma, each 1-2cm. in diameter,
outlined by thin fibrous septa. Each hexagonal area is called
a lung lobule containing lung tissue supplied by 3-5 terminal
bronchioles followed by 3-5 respiratory acini (including
respiratory bronchioles, alveolar ducts & alveolar sacs).
Normal
Lung

Lung
Lobule

containing 3-5 terminal bronchioles & 3-5 respiratory acini

Lung Lobule

=3 - 5 Respiratory Acini

Terminal bronchiole
Respiratory bronchiole
Alveolar duct
Alveoli

Respiratory Acinus

Pulmonary Emphysema

Microanatomy: The
acinus is the part of
the lung distal to the
terminal bronchiole
and includes
respiratory
bronchiole,
alveolar ducts and
alveoli.

Pulmonary Emphysema

Pulmonary Emphysema
Definition: It is characterized by
permanent enlargement of airspaces
distal to the terminal bronchiole (respiratory
bronchioles, alveolar ducts & alveoli)
accompanied by

destruction of their walls.

N.B. There are several conditions in


which enlargement of air spaces is
not accompanied by destruction; this is
more correctly termed as over inflation.

Classification of Emphysema
Emphysema is classified according to its
anatomic classification within the lobule
(cluster of acini , the alveolated respiratory
units).
There are 4 major types:
1.centriacinar, 2.panacinar, 3.paraseptal ,
and 4. irregular. Of these, only the first two
cause clinically significant obstruction.
N.B. Centriacinar is far more common than
panacinar form, constituting more than
95% of cases

Types of Emphysema
1.Centrilobular (Centriacinar) Emphysema

There is enlargement of the


respiratory bronchiole (central or
proximal part) while the distal alveoli
are spared (normal). Thus both
emphysematous & normal airspaces
exist in the same acinus and lobule. It
is common in the upper lobes &
associated with smoking.

1.Centrilobular (Centriacinar)
Emphysema
Alveolar
duct

Respiratory
bronchiole

Alveoli

2.Panlobular (Panacinar) Emphysema


There is enlargement of all the acinus
including respiratory bronchiole,
bronchiole alveolar
ducts, and alveoli.
It is more common in the lower lobes and in
the anterior margins of the lung, & usually more
severe in the base.
base
It is associated with alpha 1 antitrypsin deficiency.
The prefix pan refers to the entire acinus & not
the entire lung.

2.Panlobular (Panacinar) Emphysema

In severe cases
of centriacinar
emphysema
the distal
acinus
becomes
involved thus
the distinction
from panacinar
emphysema
becomes
difficult.
difficult

3.Distal Acinar (paraseptal)


Emphysema
The proximal portion of the acinus
(respiratory bronchiole) is normal but the
distal part is enlarged (alveolar duct &
alveoli).
alveoli) The emphysema is more striking
adjacent to the pleura (can produce
spontaneous pneumothorax in young adults),

along the lobular connective tissue septa,


septa
at the margins of the lobules. It occurs in
the upper half, near areas of fibrosis ,
scarring or atelectasis.

3.Distal Acinar (paraseptal)


Emphysema

Alveolar
ducts &
alveoli

4. Irregular Emphysema (Airspace


Enlargement with Fibrosis)
Irregular emphysema is so named
because the acinus is irregularly
involved. It is associated with scarring
& fibrosis from a healed inflammatory
process.
In most instances these foci of
emphysema are asymptomatic
(apparently healthy individuals) and
clinically insignificant.

What is the pathogenesis of


emphysema?

Pathogenesis of Emphysema
2 critical imbalances:
A. protease antiprotease) B. (Oxidant antioxidant)
Alpha 1 antitrypsin is synthesized by liver,
present in serum, tissue fluid & macrophages)
has antiprotease activity (inhibits elastolytic
activity)
Protease is secreted by neutrophils and
macrophages ( it has elastolytic activity)
O free radical inhibits the function of the alpha 1
antitrypsin ( it is present in smoke & can be
secreted by activated neutrophils)

Pathogenesis of Emphysema
If alpha 1 antitrypsin is decreased
(deficiency or functional deficiency by O
free radical)
or
If protease is increased by inflammation
(chronic bronchitis) or smoking
(chemoattraction of N & M)
Imbalance results & destruction occurs

B. Oxidant Antioxidant Imbalance


Normally the lung contains antioxidants.
Tobacco smoke contains abundant
reactive oxygen species (free radical).
Activated neutrophils also secrete
reactive O free radical which in addition
to its oxidative damage results in
inactivation of antiprotease, i.e.
functional deficiency.
This results in oxidative damage.

Pathogenesis of Emphysema

Normally neutrophils are present in capillaries including those of lung and


few pass into the alveoli (more in the lower zones than in the upper).

Any stimulus that


increases the no. of
neutrophils and
macrophages in the
lung

with

Increased protease
(elastase) & as well O2
free radical (inhibit
alpha 1 activity)

low levels of alpha 1


antitrypsin by deficiency
or functional deficiency

Unchecked
tissue
destruction

Emphysema

In smokers:
Tobacco

Pathogenesis of
Emphysema
Tobacco

Nicotine

Chemoattraction of
neutrophils from the
capillaries to the
respiratory acinus

Increase no. of
neutrophils

Reactive O2 free
radical
Inactivation of anti
-protease (functional
deficiency)=oxidative inactivation

Neutrophil
elastase &
other proteases

Tissue

destruction

*Alveolar
macrophage
secretes
macrophage
elastase&
other proteases

Pathogenesis of Emphysema
Smoke particles are
impacted at respiratory
bronchiole result in
increased influx of
neutrophils & macrophages
both of which secrete
proteases
Smoke-induced oxidative
damage (O free radical)
results in functional
deficiency of alpha
antitrypsin
This results in uncontrolled
proteolysis & destruction of elastic
tissue in centriacinar region.

Pathogenesis of Emphysema

In alpha 1 antitrypsin deficiency there is


panacinar emphysema reflecting the
decrease in alpha 1 antitrypsin through the
whole acinus = panacinus.
This type of emphysema is present in lower
lobes because more neutrophils are brought
to the lower zones due to greater perfusion
by gravity with increased proteases.
Finally, some consider the upper lobe
distribution of centriacinar emphysema also
reflects the relative lack of the normal serum
alpha1antitrypsin delivery to this less perfused
region in addition to increased N & M by
smoke (nicotine, tobacco & O2 free

1. Why emphysema in smokers is centriacinar &


2. why more in upper lobes?

1. Due to impaction of smoke particles


at the respiratory bronchioles with
subsequent attraction of neutrophils &
macrophages thus more protease
production & more destruction at this
region in addition the O free radical
causes functional inactivation of
normal alpha 1 antitrypsin.
2. The upper lobes are less perfused
by blood thus there is a relative lack of
antitrypsin (antiprotease) brought to
this area.

1. Why emphysema in alpha 1 antitrypsin deficient


patients is panacinar & 2. why more in lower lobes?

1. In alpha 1 antitrypsin deficiency there


is panacinar emphysema reflecting the
decrease in alpha 1 antitrypsin through the
whole acinus = panacinus.
2. This type of emphysema is
present in lower lobes because more
neutrophils are brought to the lower
zones due to greater perfusion by gravity
which results in increased proteases.

What are the pathological


features of emphysema?

Emphysema

Emphysema
Microscopically:
There is thinning and
destruction of alveolar walls.
The adjacent alveoli become
confluent creating large air
spaces.
Fibrosis and chronic
inflammation around
bronchioles occur with the
loss of elastic tissue in the
surrounding alveolar septa in
cases associated with chronic
bronchitis.

Emphysema & Chronic Bronchitis

What is the clinical picture?

Emphysema (Pink Puffer)


The clinical manifestations do not appear until at least one third of the lung parenchyma is damaged.
damaged

1. Progressive dyspnea but cough is often slight


2. Hyperventilation occurs with the result of well
oxygenation
3. Hyperinflation of lung with small heart
4. Massive lung collapse due to rupture of
emphysematous bullae leading to pneumothorax

Chronic Bronchitis (Blue Bloater)


1. Cough with expectoration of large amount of sputum
2. Cyanosis,
Cyanosis hypercapnea (CO2) and hypoxemia

(leading

to polycythaemia)

3. Pulmonary hypertension and later corpulmonale

Clinical changes seen in emphysema:


1. Prominent sternoclavicular
muscles due to increase use of
accessory muscles of respiration.
2. Shoulders are held high.
high
3. Barrel shaped chest and ribs are
almost horizontal.
4. Liver dullness is reduced due to
downward displacement of
diaphragm.
5. The heart is covered by lungs.

What are other conditions


related to Emphysema?

Conditions related to Emphysema


1. Compensatory emphysema refers to
compensatory dilatation of air spaces in
response to loss of lung substance
better to be called compensatory over
inflation. e.g. after lobectomy
2. Senile Emphysema refers to over
distended lungs of elders, resulting from
age related changes (large alveolar
ducts & small alveoli) but there is no
tissue destruction/ Senile over inflation.

Conditions related to Emphysema


3. Obstructive Over inflation refers to
expansion of the lung due to trapped air
resulting from partial obstruction by a tumor
or foreign body.
4. Mediastinal, interstitial, or subcutaneous
Emphysema refers to air entering in
connective tissue septa of the lung,
mediastinum ,and subcutaneous tissue.
It may occur : 1. due to sudden increase in intra
alveolar pressure e.g. with vomiting or violent
coughing or 2. due to lung injury by fractured rib
or perforating wound.

Root of the
neck

Mediastinal &
subcutaneous
(interstitial)
Emphysema
Interstitial subcutaneous
emphysema
(subcutaneous crepitation)

Fractured clavicle

Lung Collapse (Atelectasis)

Lung Collapse (Atelectasis)

Atelectasis refers either to


incomplete expansion of the
lungs (neonatal atelectasis) or to
the collapse of previously
inflated lung, producing areas of
relatively airless pulmonary
parenchyma.

Lung Collapse (Atelectasis)


Definition: It is the loss of lung volume
caused by inadequate expansion of air
spaces.
It is associated with shunting of inadequately
oxygenated blood from pulmonary arteries to veins,
resulting in ventilation perfusion imbalance &
hypoxia.
hypoxia
On the basis of underlying mechanism or the
distribution of alveolar collapse it is divided
into 3 categories: 1. compression collapse,
2. absorption collapse & 3. contraction collapse.

Lung Collapse (Atelectasis)


1. Compression Collapse
It occurs as result of
accumulation of air, fluid or
blood in the pleural cavity,
leading to pressure on the lung &
collapse. The mediastinum shifts
away of affected lung
Pathogenesis: Bronchial secretions
will be drained since there is no
obstruction. (infection is late-if
untreated)

Fate :In pleural effusion or haemothorax, organization &


fibrosis of the pleura prevents re expansion.
So drainage of the pleura is important to allow re expansion.

Lung Collapse (Atelectasis)


1. Compression Collapse

Left Pneumothorax & Lt. Lung Collapse


The mediastinum
is shifted away
from the affected
lung (the air in the
pleural cavity
collapsed the lt. lung
& pushed it & the
mediastinum to the
other side, i.e.
normal rt side)
R

1. Compression Collapse

Left pleural
effusion: The
mediastinum
is shifted
away from the
collapsed side
(Left side)
Rt is Normal, the mediastinum is shifted
to the rt.

2.Resorption( Absorption )Collapse


It occurs when bronchial obstruction prevents air
from reaching distal air spaces. The mediastinum is
shifted toward the affected lung.

Acute

complete
obstruction by:
1.foreign body
(children),
2. mucus or
mucopurulent
plug (chronic

Chronic obstruction
by 1.tumors in
bronchial wall or
pressure on the wall
2.from outside by
enlarged hilar L.N. or an
aneurysm.

bronchitis or
bronchiectasis) or
3.blood clots during
surgery.

Pathogenesis:
Pathogenesis After obstruction collateral air ventilation
may keep the obstructed segment for a time. Later air is
replaced by secretions which may lead to infection.
infection

2. Right Resorption( Absorption )Collapse

The mediastinum is shifted towards the right diseased &


collapsed lung

2. Left Resorption( Absorption )Collapse

The mediastinum is shifted towards the left diseased &


collapsed lung

Compare

Lt. tension pneumothorax


caused lt. .lung
compression collapse
Compressed the same side of lung &
shifted the mediastinum to the other
side

Lt. lung absorption collapse


due to obstruction of lt.
bronchus
The air is absorbed on the same
side & shifted to diseased lung

Compare

Rt. pleural effusion

Lt. lung collapse

Compression Collapse

Absorption Collapse
Vascular 2007

3.Contraction Collapse (Atelectasis)


It occurs when either local or generalized fibrotic
changes in the lung or pleura prevent lung
expansion & increase elastic recoil during expiration.
N.B.

Significant collapse reduces oxygenation


(hypoxemia) and predisposes to infection
(bronchitis, bronchiolitis & bronchiectasis).
bronchiectasis)
Since lung collapse is a reversible disorder
(except that caused by contraction), thus early
treatment of the cause is essential to allow
re expansion of the collapsed lung.

What are the pathologic


features of lung collapse?

Gross

Lung Collapse (Atelectasis)

1. Pleural surfaces are wrinkled.


2. The affected lobe is airless,
airless sinks in water & is
purple in colour (bluish red)
red due to reduced
haemoglobin.

Mcs
1. Slit like opening of the alveoli & pulmonary
arterioles are constricted.
2. If not treated: proliferation of pneumocytes
occur, progressive pulmonary fibrosis & intimal
fibroelastosis of pulmonary arterioles

Acute Massive Lung Collapse


It affects one or both lungs
Causes:
1.Chest wall injuries due to extensive
pneumothorax or haemopneumothorax
2.Surgical operations due to bronchial
obstruction by mucus plug
3.Abnormal elevation of diaphragm as in
severe peritonitis & subdiaphragmatic
abscess

Complete the following statements by specifying the type


of emphysema
1. occurs in apparently healthy persons,
persons in
upper half , subpleural in position and is usually
asymptomatic unless pneumothorax develops.
2. occurs mainly in lower half & is associated
with alpha 1 antitrypsin deficiency.
3. .occurs in residual lung after lobectomy.
lobectomy
4. .. may occur due to vigorous coughing in a
child suffering of whooping cough.
5. ..occurs most commonly in smokers.
smokers
6. is usually asymptomatic, discovered
accidentally & is associated with scarring.
scarring
7. occurs due to diminished elastic recoil in
elderly persons.
persons

By the end of this lecture you will able to :


Define emphysema and describe its different types.
types
Explain the pathogenesis of emphysema.
Describe the pathologic features of emphysema,
recognize its clinical picture & list its complications .
Describe the conditions that mimic emphysema.
Define lung collapse & describe its different types.
List the main characteristics of obstruction collapse
Give reasons :1.The centriacinar emphysema
occurring in smokers is more common in the upper lobe ,
while the panacinar emphysema occurring in alpha1
deficient individuals is more common in lower lobe.
lobe
2. Early treatment of lung collapse is
essential.
essential