Emphysema

Definition:
 Pulmonary emphysema, from the Greek “ to inflate or to blow into”, is an obstructive
pulmonary disease characterized by the permanent dilation and destruction of lung units
from the terminal bronchioles to the alveoli:
o Respiratory bronchioles
o Terminal respiratory bronchioles
o Alveolar ducts & sacs
o Alveoli
 Confirmation of the pathology can be made only by a tissue biopsy or autopsy; however,
clinical and diagnostic findings are highly suggestive of the disease (Chest x-ray, blood
tests, ABG’s, PFT’s, chest exam, etc.)

Emphysema is classified according to its anatomical location:

 Panlobular or panacinar emphysema involves enlargement of all airspaces distal to the
terminal bronchioles including the respiratory bronchioles, alveolar ducts, and alveoli
o “Genetic” emphysema most commonly caused by alpha-1 antitrypsin deficiency
o Alpha-1 antitrypsin is secreted by the liver. It inhibits the effects of enzymes
(proteases) that digest proteins. It blocks the effect of proteases such as elastin
and collagen which are major proteins which form the structure of the lungs.
Without alpha-1 antitrypsin, elastase is free to breakdown elastin which
contributes to the elasticity of the lung.
o Alpha-1 protease inhibitor is responsible for protecting tissues from enzymes of
inflammatory cells, especially elastase.
o Affects the lower lung fields
o May occur in people who have never smoked cigarettes
o Occurs in younger people; 3 in 4 of adults with alpha-1 antitrypsin deficiency
will develop emphysema on or before age 40. Cigarette smoking will hasten the
development of the disease.

 Centrilobular or centriacinar emphysema primarily involves the central acinar respiratory

bronchioles, enlarged due to destruction of shared walls thus sparing distal lung units.
o Upper lung fields – almost always with chronic bronchitis
o Rarely occurs in non-smokers (second hand smoke can be a factor)
o Most common type of emphysema
o Occurs in older patients
Etiology:
 Two main factors:
o Cigarette smoking
o Alpha-1 antitrypsin deficiency
 Smoking increases protease activity which destroys the terminal bronchioles and alveolar
walls. It also slows mucociliary transport, leading to retention of secretions and
increased susceptibility to pulmonary infections.
 A small number of individuals develop emphysema with minimal or no smoking
exposure as a result of alpha-1 antitrypsin deficiency.
 Recurrent pulmonary infections in childhood may result in the development of an
obstructive pulmonary disease in later life.
  Exacerbation of the disease may result when pollutant levels are high. So, patients with
COPD should avoid infections and inhaled irritant in order to prevent exacerbations.
Pathophysiology:
 Because of the tissue destruction and loss of elastic recoil that occurs in emphysema,
limitations in exhaled flow and abnormalities in gas exchange exist.
 Decreased flows during forced exhalation and an increase in FRC, RV, and TCL
 Destruction of the alveoli results in reduced surface area, and airway dilation increases
the distance for gaseous diffusion. This abnormality causes ventilation/perfusion
mismatching and contributes to the increase in the work of breathing (WOB). Excess
ventilation (air trapping) to perfusion occurs.

Clinical Manifestations:
 Medical History
o Emphysema usually occurs in conjunction with chronic bronchitis
o Pink Puffer – emphysema patients have mild to moderate hypoxemia so they are
not as cyanotic as chronic bronchitis patients; therefore, they are “pink” and use
pursed-lip breathing which gives them this name
o Blue Bloater – chronic bronchitis patients have moderate to severe chronic
hypoxemia with cyanosis. Due to the cor pulmonale or right heart failure, there
is edema giving these patients a bloating effect.
 Often the patient complains of shortness of breath (SOB) increasing during exercise or
activity. Dyspnea at rest occurs later in the disease process.
 Exacerbations of the disease occur, most commonly, after:
o Infection
o Exposure to air pollutants
 A history of cigarette smoking should alert the physician that the patient may develop not
only emphysema but also other smoking-related diseases such as chronic bronchitis and
heart disease.
 A diagnosis of alpha-1 antitrypsin deficiency should also alert the physician that
emphysema may occur early in life.

Medical Examination:

 May provide key information of the diagnosis.

 Inspection of the chest will demonstrate:
o Barrel chest (increased A-P Diameter)
o Use of accessory muscles
o Retractions
o Increased expiratory time
o Abdominal paradox (inward movement of the abdominal wall during inspiration)
 Body position – leaning forward with their hands braced on their knees or their elbows
braced on a table (tripod position). This position provides more optimal mechanical
advantage for their respiratory musculature.
 Palpation reveals decreased tactile and vocal fremitus
 Percussion reveals flattened, immobile hemi diaphragms and increased resonance over
the lung fields
 Auscultation reveals decreased breath sounds and decreased voice sounds.
Laboratory Evaluation:
 Chest x-ray shows decreased vascular markings, hyper expanded lung fields with low,
flattened diaphragms, and a small, vertically oriented heart
o The lateral chest view shows an increased retrosternal space
 PFT’s (not routinely performed during an exacerbation)
o Increased RV, FRC, TLC due to air trapping
o FVC and FEV1 are decreased due to airflow obstruction
 ABG’s show normal acid-base balance with moderate hypoxemia in mild to moderate
emphysema and the development of respiratory acidosis with more severe hypoxemia,
which occurs during the terminal stage of the disease.

Management:
 Acute and supportive care
o Increased use of bronchodilators
o Systemic corticosteroids
 Long-term care is designed to:
o Prevent progression (stop smoking)
o Enhance survival (home oxygen)
 Pulmonary Rehabilitation Goals:
o Patient and family education, counselling & support
o Improve ventilatory and cardiac status
o Improve ambulation and other physical activities
o Minimize respiratory infections
o Decrease number of hospitalizations
 Stop smoking – the patient must be counseled on the importance of not smoking and the
need to avoid exposure to respiratory tract infections and irritants
 Patient should be encouraged to receive the flu and pneumonia vaccines annually, as an
attack of the flu will precipitate an exacerbation requiring admission to the hospital.
 Proper nutritional status must also be emphasized (these patients may require up to 10
times the calories a healthy person needs to breathe):
o Low salt diet to decrease water retention
o Low caffeine diet as caffeine causes restlessness & anxiety
o Avoidance of foods that cause gas or bloating
 Supplemental oxygen is needed when the resting room air PaO2 is less than 55 mmHg
o Nasal cannula at 1-2 lpm should be enough (if an acute exacerbation is
precipitated by an acute pneumonia or congestive heart failure, hypoxemia may
be severe and require more significant elevation of FIO 2 )
 Bronchodilators are important agents for the treatment of bronchospasm when associated
with exacerbation of emphysema.
 The administration of antibiotics is indicated when a respiratory tract infection is present,
typically seen as a change in sputum quantity, color, and /or consistency (yellow)
 Good hydration is needed to improve pulmonary hygiene
 Acute respiratory failure may require continuous mechanical ventilation only when a
result of a reversible problem is superimposed on the patient’s COPD