* Dr. S. Y.

So
M.B. B.S., ( H K ) ,
M.R.C.P. ( UK ).

abnormal, permanent enlargement of the

Pulmonary Emphysema
A major public health problem
nowadays is chronic obstructive pulmonary
diseases such as emphysema. It is responsi-
ble for much work-loss and hospital admis-
sions above the age of 40 and it is a
common cause of death. The problem is
getting worse since no active effective
measures are carried out to combat smok-
ing and'atmospheric pollution.
Definition
Pulmonary emphysema is defined as
a condition of the lung characterised by
air spaces distal to the terminal bronchiole
accompanied by destruction of their walls.
The definition, given in pathological terms,
separates
(1) Normal enlargement of the air spaces
due to aging, and
(2) Enlargement without destruction of
alveolar walls as in compensatory
overinflation.
Pathology
There are four pathological types of
emphysema (Fig. 1). Their salient features
are listed in Table 1. Discussion here is
confined to those with generalised
panacinar emphysema.

Table 1

Anatomical Types of Emphysema

Panacinar Centriacinar Periacinar Irregular

( Panlobular ) ( Centrilobular ) ( Paraseptal ) (Scar)

Involvement . Whole acinus Central region Edge of Irregular

of acinus acinus

Sex : Men & Women Men more Men more Men & women

Distribution in lungs : Mainly lower Mainly upper Upper zones Variable

zones zones

Chest radiographic Apparent Not apparent Bleb Variable

changes:

Airflow obstruction Due to airway Due to intrinsic Nil Variable

collapse airway disease

Clinical syndrome : ''Pink puffer" "Blue bloater" Spontaneous Underlying

pneumothorax disease
in the young

* Lecturer in Medicine, University of Hong Kong.

57
 continued
Pathogenesis
During inflammation, polymorphon-
uclear leukocytes and alveolar macrophages
release proteolytic enzymes which may
destroy alveolar walls leading to emphy-
sema. However these enzymes are normally
inhibited by a, globulin e.g."i-antitrypsin.
It is conceivable that emphysema may arise
either due to
(1) overproduction or oversecretion of
preteolytic enzymes
e.g. by chronic smoking or
(2) deficiency of enzyme inhibitor
e.g. familialai -antitrypsin deficiency
with accounts for about 5% of
patients with emphysema.
(B)
Diagnosis in life
Since an accurate diagnosis of emph-
ysema could only be made at autopsy, its
recognition in life is often imprecise. How-
ever the followings may help
(A) Clinical Features
Patients are typically in their 60's
when first seen ( except those with
atantitrypsin deficiency when they
present at around 40 ). Progressive
exertional dyspnoea is the chief
complaint. There is little cough and
sputum. Cor pulmonale is only a
terminal event. A history of weight
loss is often present. On examination
they are usually thin, intensely
dyspnoeic with pursed-lip breathing
and not cyanotic. Dornhorst there-
fore called them as "pink puffers".
Chest examination reveals two
prominent features, namely,
58
1. hyperinflation of the chest, with
barrel chest deformity, high shoulder
prominent accessory muscles, wide
subcostal angle, diminished chest
expansion, hyper-resonance on per-
cussion with diminished cardiac and
liver dullness, and faint breath
sounds and heart sounds.
2. signs of diffuse airflow obstruction
with prolongation of expiratory
sound.
Patients often die from respiratory
failure with co-existing respiratory
tract infection. Spontaneous pneu-
mothorax occurs occasionally.
Radiological Features
These include
/. Hyperinflation of chest. This shows
as a low, flat diaphragm - below the
6th rib anteriorly - with a large
retrosternal translucent area.
2. Cardiovascular changes. These
include a narrow vertical heart with
prominent pulmonary trunk and
large hilar vessels, together with
small lung vessels at the periphery.
3. Bullae. These show as avascular
transradiant areas which are either
priorly demarcated or are demarcat-
ed by a white line.
If the first two of the above three
signs are present, severe emphysema
is likely. If hyperinflation of chest
alone is seen, asthma should be
excluded. Mild panacinar emphy-
sema may be associated with a
normal chest radiograph.
 continued
(C) Lung function tests. If these show ir-
reversible airflow obstruction togeth-
er with a low gas transfer of carbon
monoxide, emphysema is very likely.
1. Airflow obstruction. Because of
alveolar destruction, there is loss
both of elastic recoil of the lung and
of supporting network for airways.
So, on expiration, intrathoracic
pressure may exceed intraluminal
pressure leading to collapse of
unsupported airways, and hence
airflow obstruction. Hyperinflation
of chest if due to a combination of
airflow obstruction and loss of
elastic recoil of the lung. Since air-
flow obstruction is due to airway
collapse, it will change little with
bronchodilator therapy. A simple
way of assessing airflow obstruction
is to measure peak flow rate with the
inexpensive Mini-Weight peak flow
metre. Alternatively a spirometer can
be used to measure forced expiratory
volume in one second (FEV-] ) and
forced vital capacity (FVC). The
ratio of FEV-j/FVC is less than 75%
in airflow obstruction.
2. Low gas transfer of carbon monoxide.
This is due to a loss of effective sur-
face available for gas exchange result-
ing from alveolar destruction and
obliteration of pulmonary capillaries.
Unlike chronic bronchitis, emphy-
sema is often associated with normal
arterial blood gases at rest, because blood
supply in damaged areas is reduced in
proportion to the reduced ventilation to
these areas. Arterial P02, however, drops
during exercise.
Management
Since damage to alveoli has already
been done, effective treatment cannot be
expected. However every effort should be
made to rehabilitate patients so that they
can lead a useful independent life.
(A) Reduction of tor flow obstruction to
decrease the worfc of'breathing.
1. Bronchodilators. Although airflow
obstruction changes little with bron-
chodilator therapy, even the smallest
increase in ventilatory capacity can
be of benefit to the disabled patient.
The drug of choice is a (02 adrenergic
stimulant e.g. salbutamol or terbuta-
line, given in the form of pressurised
aerosol, for greater efficacy and less
cardiac side effects. An anticholiner-
gic agent, ipratropium bromide, may
also help. Theophyline derivatives
vary in their effectiveness because of
wide differences in drug metabolism
among patients. Steroids should be
used only when there is demonstra-
ble objective improvement since a
state of euphoria not uncommonly
follows after therapy.
2. Reduction of bronchial secretions
Sputum production is a problem
when there is co-existing chronic
bronchitis. Cessation of smoking and
avoidance of pollution often help.
The value of expectorants and muco-
lytic agents is controversial. Mainten-
ance of adequate hydration is more
important if viscous secretion is to
be avoided.
59
 Continued
3. Control of infection
Bacterial infection often complicates
the desease and is due to H. influen-
zae or S. pneumoniae. Ampicillin or
Tetracycline is the drug of choice.
(B) Physical Therapy
1. Breathing Retraining. Patients often
breathe inefficiently with a rapid
shallow pattern. The goal of breath-
ing retraining is to teach patients to
breathe more deeply and slowly,
especially during expiration. This
will reduce the work of breathing
and patients often feel better althou-
gh there is little change in pulmonary
function tests.
2. Pursed-lip breathing. This will
increase intra-airway pressure during
expiration so that airway collapse is
prevented. Many patients breathe
this way intuitively.
3. Exercise-Reconditioning. Patients
should be encouraged to remain
active. They should gradually increa-
se the duration and speed of walking.
After reconditioning, pulmonary
function tests are not much altered
but patients often feel and function
better because of more effective
utilization of oxygen by exercising
muscles.
4. Oxygen-supported exercise. Oxygen
is indicated when there is demonstra-
ble improvement in exercise toleran-
ce or a decrease in heart rate at a
60
given exercise level. It can beadmini-
tered via nasal prongs from the
conventional oxygen tank, liquid
oxygen system, or the new oxygen
concentrator using electronic mole-
cular sieves that remove nitrogen
from the air. The aim is to keep Pa02
at 60 mmHg at rest and also during
exercise. This can usually be done at
a flow rate of about 2-3 litres/min at
rest and 4-5 litres/min during exer-
cise. No oxygen-related accidents
have been reported in patients with
low-flow home oxygen. The cost of
oxygen for 1 month at 2 litres/min
is around $400.
(C) Surgical removal of large bullae.
This only benefits the occasional
patient who remains quite disabled
despite the above measures. Improve-
ment after surgery is thought to be
due to re-expansion of areas of lung
previously compressed by bullae.
Careful pre-operative regional lung
function tests are necessary and not
all patients do well after surgery.
(D) Psychosocial support.
This is necessary for someone who
is disabled by such a chronic disease.
Please refer to next page
for diagrammatic illustration
 continued

Legends

Diagrammatic representation of various anatomical types of emphysema.

.. ;. •• •. «*B • .- -. .• ...

*• * •*•/•"• «^ ' •**• **

A - Panacinar emphysema. 1 - Lobular bronchiolus.

B - Centriacinar emphysema. 2 - Terminal bronchiolus.
C - Paraseptal emphysema. 3 - Respiratory bronchiolus.
D - Irregular emphysema. 4 - Septum.
5 - Pleura.