Emphysema

Author: Tanner Marshall, MS, Robert M. Leven, PhD

Editor: Rishi Desai, MD, MPH

Emphysema means inflate or swell, which makes sense because in the lungs of people with
emphysema, the alveolar air sacs, which are the thin walled air spaces at the ends of the
airways where oxygen and carbon dioxide are exchanged, become damaged or destroyed. The
alveoli permanently enlarge and lose elasticity, and as a result, individuals with emphysema
typically have difficulty with exhaling, which depends heavily on the ability of lungs to recoil like
elastic bands.

Emphysemas actually lumped under the umbrella of chronic obstructive pulmonary disease (or
COPD), along with chronic bronchitis. They two differ in that chronic bronchitis is defined by
clinical features, like the productive cough, whereas emphysema is defined by structural

changes, mainly enlargement of the air spaces.

That being said, they almost always coexist, probably because they share the same major
causesmoking.

With COPD, the airways become obstructed, the lungs dont empty properly, and that leaves air
trapped inside the lungs. For that reason, the maximum amount of air people with COPD can
breath out in a single breath, known as the FVC, or f orced vital capacity, is lower. This reduction
is especially noticeable in the first second of air breathed out in a single breath, called

FEV1forced expiratory volume (in one second), which typically is reduced even more than the
FVC.

A useful metric therefore is the FEV1 to FVC ratio, which, since the FEV1 goes down even more
than FVC, causes the FEV1 to FVC ratio to go down as well.

Alright so say normally your FVC is 5 L, and your FEV1 is 4 L, your FEV1 to FVC ratio would end
up being 80%. Now, someone with COPDs FVC might be 4 L instead, which is lower than

normal, but the volume of air that he or she can expire in the first second is only 2 L, so not only
are both these values lower, but their ratio is lower as welland this is a hallmark of COPD.

All that had to do with air breathed out right? Conversely, for air going in, the TLC, or total lung
capacity, which is the maximum volume of air that can be taken in or inspired into the lungs, is
actually often often higher because of the air trapping.

Alright, so emphysema is a form of COPD, thats based on structural changes in the lung,
specifically a destruction of the alveoli.

Normally, though oxygen flows out of the alveoli and into the blood while carbon dioxide makes
the reverse commute, but when the lung tissue is exposed to irritants like cigarette smoke, it
triggers an inflammatory reaction that upsets the delicate alveolar walls and affects the flow of
gases. Inflammatory reactions attract various immune cells which release inflammatory
chemicals like leukotriene B4, IL-8, and TNF alpha, as well as proteases, like elastases and
collagenases. These proteases break down key structural proteins in the connective tissue layer
like collagen, as well as elastin, which is a protein that gives the tissue elasticity, and this leads
to the problems seen in emphysema.

In healthy lungs, during exhalation, air whizzes through the airways with high velocity, creating a
low pressure environment in the airway. This is due to the Bernoulli principle, where, as a
fluidwhich includes airmoves at higher velocity, it must have lower pressure. Now this lower
pressure tends to pull the tiny airway inward. Strong healthy airway walls full of elastin can
withstand that pressure and dont collapse; they hold the airway open and allow air to fully
escape during exhalation.

With emphysema though, that elastins lost, which makes the airway walls weak and allow that
low pressure system to pull the walls inward and collapse during exhalation.

This ultimately leads to air-trapping because the collapsed airway traps a tiny bit of air distal to
the point of collapse. Also, this loss of elastin makes the lungs more compliant, meaning that
when air blows into them, they easily expand and then hold onto that air instead of expelling it
during exhalation, and so the lungs start to look like large thin plastic bags.

The loss of elastin also leads to a breakdown of the thin alveolar walls called septa. Without
these walls, neighboring alveoli coalesce into larger and larger air spaces, which means the

surface area available for gas exchange is reduced (relative to the expanding volume), which
affects oxygen and carbon dioxide levels.

This process all happens in the acinus, which is the endings of the lung airways where those
clusters of alveoli are located. Different types of emphysema affect the acinus slightly differently.
The first pattern of emphysema is called c entriacinar emphysema, or centrilobular emphysema,
and this is the most common pattern and it really only damages the central or proximal alveoli of
the acinus. This is the pattern seen with cigarette smoking and is thought to happen because
the irritants from smoke arent able to make it all the way to the distal alveoli. Centriacinar
emphysema typically affects the upper lobes of the lungs.

There is also panacinar emphysema, where the entire acinus is uniformly affected, and this is
often associated with the genetic condition alpha-1 antitrypsin deficiency.

Now in healthy individuals, macrophages are always letting out some proteases to help clear
the debris that occasionally finds its way into the acini, but those proteases break down
proteins, right? So these can damage the tissue. Alpha-1 antitrypsin is a protease i nhibitor
generated by the body, to protect against unintended collateral damage from the proteases.
People with alpha-1 antitrypsin deficiency dont have these protective proteases inhibitors, and

so they end up with damaged air sacs, that affect the entire acinus. Panacinar emphysema
typically affects the lower lobes of the lungs.

A third and final type of emphysema is called paraseptal emphysema in which the distal alveoli
of the acinus are most affected, and this type typically affects the lung tissue on the periphery of
the lobules, near the interlobular septa, that separate each lobule. The thing to keep in mind
about paraseptal emphysema is that the ballooned out alveoli on the lung surface can rupture
and cause a pneumothorax.

People with emphysema typically experience symptoms like dyspnea, which is a shortness of
breath, due to the air trapping and diminished gas exchange. To help counteract this, people
sometimes exhale slowly through pursed lips, which increases pressure inside the airways and
preventing them from collapsing as easily. This way of breathing explains the nickname pink
puffers, since individuals are able to oxygenate their blood, but have to purse their lips to do so.
All of this constant energy spent on breathing can even cause weight loss. Over time, though,
as more and more lung tissue is affected, emphysema can lead to hypoxemia, or low oxygen in
the blood. There can also be a cough with a small amount of sputum from inflammation in the
small bronchioles that causes excess mucus production via goblet cells, but this is a lot different
from the productive cough with lots of sputum seen in chronic bronchitis.

Over time, air-trapping and hyperinflation of the lungs can cause individuals to develop a
barrel-shaped chest, and on x-ray, individuals might have an increased anterior-posterior
diameter, a flattened diaphragm, and increased lung field lucency.

Alright, so in normal physiology there is a process called hypoxic vasoconstriction, where if, for
some reason, one area of the lungs has poor gas exchanges, then the blood vessels going to
that area undergoes vasoconstriction in an attempt to shunt blood to an area with better gas
exchange. This works great if the hypoxia is localized to one area of the lungs, but when a large
proportion of the lungs arent exchanging oxygen effectively, then that vasoconstriction starts
involving too many blood vessels and this leads to pulmonary hypertension. Over time, this
increases the work that has to be done by the right side of the heart to pump blood to the lungs,
causing it to enlarge, a process called cor pulmonale, which eventually to right-sided heart
failure.

Treatment of emphysema largely involves reducing risk factors and managing associated
illnesses. Since smokings a major player in c ausing emphysema, stopping smoking is a major
player in reducing mortality. Supplemental oxygen, as well as certain medications like
bronchodilators, inhaled steroids, and antibiotics to control secondary infections are all helpful in
managing emphysema.

Alright as a quick recap, emphysema is a type of chronic obstructive pulmonary disease or

COPD, where where exposure to irritantslike smokingcauses elastin in the small airways
and alveolar walls to be broken down, and this leads to air trapping and poor gas exchange,
both of which eventually lead to hypoxemia.

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Sources:
http://emedicine.medscape.com/article/298283-overview#a5
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Pathoma (text)
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https://en.wikipedia.org/wiki/Chronic_obstructive_pulmonary_disease