Case 6: Learning Objectives

1. Develop a differential diagnosis for acute onset dyspnea.

Causes of acute onset dyspnea: pulmonary disease, cardiac disease, acute blood loss, metabolic acidosis,
anxiety, and poor physical condition. Pulmonary diseases include bronchitis, emphysema, asthma,
pneumonia, and pneumothorax are typical causes of acute dyspnea.
Acute bronchitis is characterized by the development of a cough, with or without the production of
sputum, mucus that is expectorated (coughed up) from the respiratory tract. Acute bronchitis often occurs
during the course of an acute viral illness such as the common cold or influenza. Viruses cause about 90%
of cases of acute bronchitis while bacteria account for less than 10%.
Chronic bronchitis, a type of COPD, is characterized by the presence of a productive cough that lasts
for 3 months or more per year for at least 2 years. Chronic bronchitis most often develops due to recurrent
injury to the airways caused by inhaled irritants. Cigarette smoking is the most common cause, followed
by air pollution and occupational exposure to irritants.
Emphysema is a lung disease, characterized by an abnormal, permanent enlargement of air spaces
distal to the terminal bronchioles. The disease is coupled with the destruction of walls, but without obvious
fibrosis. It is often caused by exposure to toxic chemicals, including long-term exposure to tobacco smoke.
Emphysema is characterized by loss of elasticity (increased pulmonary compliance) of the lung tissue
caused by destruction of structures feeding the alveoli, in some cases owing to the action of alpha 1antitrypsin deficiency. This causes the small airways to collapse during forced exhalation, as alveolar
collapsibility has decreased. As a result, airflow is impeded and air becomes trapped in the lungs, in the
same way as other obstructive lung diseases. Symptoms include SOB on exertion, and an expanded chest.
However, the constriction of air passages isn't always immediately deadly, and treatment is available.
Asthma is a predisposition to chronic inflammation of the lungs in which the airways (bronchi) are
reversibly narrowed. During asthma attacks (exacerbations of asthma), the smooth muscle cells in the
bronchi constrict, and the airways become inflamed and swollen causing difficulty breathing.
Pneumonia is an inflammatory illness of the lung. Frequently, it is described as lung parenchyma/alveolar
inflammation and abnormal alveolar filling with fluid (consolidation and exudation). Pneumonia can result
from a variety of causes, including infection and chemical or physical injury to the lungs.
A pneumothorax is a potential medical emergency wherein air or gas is present in the pleural cavity. A
pneumothorax can occur spontaneously. It can also occur as the result of disease or injury to the lung, or
due to a puncture to the chest wall. A pneumothorax can result in a collapsed lung, or can be created
therapeutically to collapse a lung.
Heart problems - including:
Irregular heart beats
Fluid accumulation around the heart due to certain forms of cancer (pericardial effusion)
A recent heart attack which may be blocking blood flow
2. Discuss the etiology of COPD.
COPD comprises chronic obstructive bronchitis (clinically defined) and emphysema (pathologically
defined). Many patients have features of both.
Etiology
Cigarette smoking is the primary risk factor, although only about 15% of smokers develop clinically
apparent COPD; an exposure history of 40 or more pack-years is especially predictive. Smokers with
preexisting airway reactivity, even in the absence of clinical asthma, are at greater risk of developing COPD.
Genetic factors The best-defined genetic disorder is 1-antitrypsin deficiency, which is an important cause
of emphysema in nonsmokers and influences susceptibility to disease in smokers.
Inhalational exposures trigger an inflammatory response in airways and alveoli that leads to disease in
genetically susceptible people. The process is thought to be mediated by an increase in protease activity and
a decrease in antiprotease activity. Lung proteases (neutrophil elastase, matrix metalloproteinases, and
cathepsins) break down elastin and connective tissue in the normal process of tissue repair. Their activity is
balanced by antiproteases, such as 1-antitrypsin, airway epitheliumderived secretory leukoproteinase

inhibitor, elafin, and matrix metalloproteinase tissue inhibitor. In people with COPD, activated neutrophils and
other inflammatory cells release proteases as part of the inflammatory process; protease activity exceeds
antiprotease activity, and tissue destruction and mucus hypersecretion result. Neutrophil and macrophage
activation also leads to accumulation of free radicals, superoxide anions, and hydrogen peroxide, which inhibit
antiproteases and cause bronchoconstriction, mucosal edema, and mucous hypersecretion. Neutrophilinduced oxidative damage, release of profibrotic neuropeptides (eg, bombesin), and reduced levels of
vascular endothelial growth factor may contribute to apoptotic destruction of lung parenchyma. Infection, in
conjunction with cigarette smoking, may amplify progression of lung destruction. The inflammation in COPD
increases with increasing disease severity, and, in severe (advanced) disease, inflammation may not resolve
completely with smoking cessation. Neither does this inflammation appear responsive to corticosteroids.
Bacteria, especially Haemophilus influenzae , colonize the normally sterile lower airways of about 30% of
patients with COPD. In more severely affected patients (eg, those with previous hospitalizations),
Pseudomonas aeruginosa colonization is common. Smoking and airflow obstruction may lead to impaired
mucus clearance in lower airways, which predisposes to infection. Repeated bouts of infection increase the
inflammatory burden that hastens disease progression. There is no evidence, however, that long-term use of
antibiotics slows the progression of COPD in susceptible smokers.

3. Compare and contrast the clinical presentation of chronic bronchitis and emphysema
Chronic bronchitis: presence of long-term cough + sputum production on most days over 3month
period for >2 consecutive years in a patient without other explanations for cough. Almost all
patients are smokers, except a small number who have chronic exposure to and airway inflammation due
to other fumes or dusts. Because of the high prevalence of smoking, chronic bronchitis remains one of the
most frequent causes of chronic cough. However, most smokers with chronic bronchitis do not seek
medical attention for their cough, and in most series of chronic cough, chronic bronchitis accounts for 5
percent or less of cases. The sputum produced is usually clear or white. A purulent appearance to
sputum often suggests a concomitant upper or lower respiratory infection, such as acute bronchitis,
bronchiectasis, or sinusitis. In any smoker who presents for evaluation of cough, one must ensure
that the symptoms do not represent a change in a chronic cough that is suggestive of a
neoplasm.
Emphysema: destruction of the lungs over time, gradual SOB, which causes pts to avoid certain
activities, affects daily activities, until SOB at rest.
4. Describe the role of PFTs in the Dx of COPD
The diagnosis of COPD is supported by finding of persistent airway obstruction on a postbronchodilator
spirometry test. In a Pulmonary Function Test (PFT) performed by a respiratory therapist, the pt.
exhales into a spirometry tube thats attached to a computer that measures several parameters of the pts
lung function (functional residual capacity, forced vital capacity, forced expiratory volume in 1 second, forced
expiratory volume in 2 seconds, total lung capacity, and residual volume, etc). COPD is defined by a
FEV1/FVC that is less than predicted for the subject. Lung volume measurements may reveal an
increased residual volume and total lung capacity though the diagnosis of airway obstruction is only made by
demonstrating an abnormality in the FEV1/FVC (ie <0.7 is cut-off value for Dx) . The diffusing capacity for
carbon monoxide is usually reduced with emphysema but preserved in patients with chronic bronchitis.
Pulmonary function generally declines progressively, and although less accurately predictable in a given
patient, the average yearly loss in FEV1 is 50 to 100 mL. The loss of FEV1 is accelerated in patients who
continue to smoke. Activity is markedly limited when the FEV1 is about 1.0 L. The postbronchodilator
FEV1, performance on a 6-minute walk, level of dyspnea, and body mass index (BMI) have been
identified as predictors of survival.
5. Discuss long-term therapy for COPD:
The management of COPD is based on the pts FEV1, and must be adjusted as the condition progresses. The
main interventions for a pt. w/ hypoxemia at rest to start with, which also happen to be proven to improve
survival rates, are smoking cessation and long-term O2 therapy (LTOT). Then, on an annual basis, pt.
should receive influenza vaccination in the early fall. Pneumococcal vaccination should also be
performed no less than once every 5 years, however, higher risk individuals should receive the injection more
frequently based on titer levels. Long-term therapy of COPD should also include use of a combination of
drugs to manage symptoms and maximize pts lung functioning. Drugs frequently used are bronchodilators

(ie short and long-acting beta2 agonists like Albuterol and Salmeterol, short and long-acting anticholinergics
like Ipratropium and Tiotropium), methylxanthines like Theophylline (last resort drug), inhaled
corticosteroids for regular use and oral or I.V. for acute exacerbations, and antibiotics at the first sign of
infection. Pt. may also benefit from pulmonary rehabilitation which is a comprehensive program of
exercise and education concerning the day to day management of their disease. As mentioned earlier, LTOT
is also used to improve quality of life and longevity. Sometimes surgeries such as bullectomy, lung volume
reduction, and even lung transplantation are required for very severe cases.
6. Discuss treatment for acute exacerbations of COPD
Exacerbation of chronic obstructive pulmonary disease (COPD) is defined as an acute increase in
symptoms (cough, sputum production, dyspnea) beyond normal day-to-day variation. Tachypnea
and decreased pulmonary function also usually present.
Goals of treatment:
Identify cause (most infection, some environmental, idiopathic)
Optimize lung function by administering bronchodilators
Assure adequate oxygen and secretion clearance
Avoid intubation if possible
Prevent complications of immobility
Pharm Major treatment is pharmacological. Treat with inhaled bronchodilators (beta adonergic agonist
Albuterol or anticholinergic agents Ipratropium Bromide), glucocorticoids (Oral: Prednisone, IV:
Methylprednisone) and Antibiotics if there is infection, for COPD choose between amoxicillin w/
clavulonic acid or fluoroquinolones.
Oxygen Therapy Aim for PaO2 of 60-70 (to maintain drive) with a saturation of 90-95%. A high FiO2 (Fraction of Inspired
O2) is not needed to correct hypoxemia associated with acute exacerbations of COPD, so if a low fraction is
not working suspect pulmonary embolism, ARDS, pulmonary edema or pneumonia.
Follow-Up Once the patient begins to improve, measures should be taken to prevent future exacerbations, including
smoking cessation, pulmonary rehabilitation, proper use of medications (including metered dose inhaler
technique), and vaccination.

7. Interpret ABGs including evaluating for the appropriateness of compensatory mechanisms.

Main blood gases: pH, PaO2, PaCO2, HCO3. They are used to determine how well you are moving O2 into
blood and how well you are removing CO2 from blood.
pH - Normal is about 7.35-7.45. Alkalosis is above this and acidosis is below this.
HCO3 - Bicarbonate. Bodies main buffering system to make sure blood pH is normal and neither too basic or
acidic.
PaO2 - The amount of oxygen that diffuses into the plasma (not bound to hemoglobin). Measured using
arterial blood. Less than 80 mm Hg is considered abnormal.
PaCO2 - The partial pressure of CO2 in the plasma. 35-45 mm Hg considered normal.
First look at pH and determine if your patient is in acidosis or alkalosis. Next you will look at PaCO2 to
determine if this state is due to respiratory or metabolic causes.
CO2 is transported in the blood as Bicarbonate in the equlibrium equation given here:
CO2 + HOH <=> H2CO3 <=> H+ + HCO3Thus, an increase in PaCO2 will drive the reaction to the right causing an increase in hydrogen ions and thus,
a decrease in pH. This is known as respiratory acidosis. It is usually due to hypoventilation (slow breathing)
or obstructions in gas exchange (CO2 cannot leave the blood) due to conditions like emphysema, asthma,
bronchitis, pneumonia and pulmonary edema. When this is the case, the method of compensation is going to
be via the kidney. Refer to handout from class for specific numbers, but basically there will be a rise in bicarb
that will somewhat offset the rise in PaCO2.

In the opposite case, hyperventilation (anxiety, drugs, high altitude, fever, pneumonia (where hypoxic drive
over rides the CO2 levels), there will be respiratory alkalosis and the compensatory mechanism will be a fall
in bicarb via the kidney.
8. Describe typical radiological findings in COPD
The chest radiograph (CXR) may reveal lung hyperinflation, flat diaphragms, a narrow or elongated heart
shadow, and bullous disease (=single or multiple large cystic alveolar dilatations of lung tissue) on a frontal
view. On a lateral projection an increased A-P diameter can be noted, indicating a barrel chest, and an
increased retrosternal air space can be observed as well. Chest X-rays are useful to help exclude other lung
diseases. However, the chest radiograph may be normal in the early stages of the disease and is not a
sensitive test for the diagnosis of COPD.
Emphysematous changes are more easily seen on a computed tomography scan of the chest but this is not
a cost-effective or recommended modality for screening COPD. Nonetheless, although imaging can suggest
the presence of COPD, only spirometry is the criterion standard for the diagnosis of airway obstruction.
Arterial blood gas measurement is recommended when the FEV1 is below 40% of predicted for the subject,
with evidence of cor pulmonale and during severe acute exacerbations to assess not just oxygenation but
also possible hypercapnia.
Testing for alpha-1-antitrypsin is recommended for patients who are diagnosed to have COPD at a young
age (<45 years old) or have strong familial history of obstructive lung disease.
9. List immunizations that patients with COPD should receive
Patients with COPD should discuss influenza and pneumoccocal immunization (vaccination) with their
physicians. In some cases, immunization is not recommended, but these vaccines are generally safe.
Influenza (the flu) is a respiratory illness that can be devastating to a patient with COPD. Vaccination
decreases the incidence of influenza significantly but does not provide 100% protection. Influenza
immunization should be given yearly, generally in early autumn.
Streptococcus pneumoniae is the most common cause of bacterial pneumonia in COPD patients among the
elderly. (Haemophilus influenza is more common in younger patients.) Vaccination can significantly decrease
the incidence of pneumonia and therefore recommended. Pneumococcal immunization (Pneumovax) is
given every 5 to 6 years since immunity wanes after about 5 years.
10. Discuss the importance of smoking cessation in the patient with COPD
COPD is caused by noxious particles or gas, most commonly from tobacco smoking, which trigger an
abnormal inflammatory response in the lung. The inflammatory response in the larger airways is known as
chronic bronchitis, which is diagnosed clinically when people regularly cough up sputum. In the alveoli, the
inflammatory response causes destruction of the tissues of the lung, a process known as emphysema.Tobacco
smoking is currently the primary risk factor for COPD in developed countries. The only interventions that have
so far been proven to improve survival in COPD patients with significant hypoxemia at rest are smoking
cessation and long-term oxygen therapy (LTOT). Thus, all patients with COPD should be encouraged to stop
smoking. Smoking cessation is one of the most important factors in slowing down the progression of COPD.
Even at a late stage of the disease it can significantly reduce the rate of deterioration in lung function and
delay the onset of disability and death. The chance of successfully stopping smoking can be greatly improved
through social support, engagement in a smoking cessation program and the use of drugs such as nicotine
replacement therapy, bupropion and varenicline
Vascular anomalies of the affected arm such as an axillary artery aneurysm (in unilateral clubbing

11. Discuss familial alpha-1-antitrypsin deficiencys role in the development of emphysema

A1A is a serine protease inhibitor, protecting tissues from enzymes of inflammatory cells, especially elastase.
Patients with alpha 1-antitrypsin deficiency (A1AD) are more likely to suffer from emphysema as the
deficiency allows inflammatory enzymes to destroy the alveolar tissue. Most A1AD patients do not develop

clinically significant emphysema, but smoking and severely decreased A1AT levels can cause emphysema at
a young age. A1AD causes about 2% of all emphysema. A1AD is panacinar damage.
12. Discuss oxygen therapy in COPD including modes of delivery, goals, risks
Oxygen is given through a flexible plastic tube inserted in the nostrils (nasal cannula) or through a face mask.
The nasal cannula gives you the greatest freedom for moving around and talking. But this method may be
more expensive than other devices because of inefficient oxygen delivery. People who need a higher flow of
oxygen can use a face mask. But a face mask is less portable and gets in the way of talking and eating.
Oxygen is usually prescribed to raise the PaO2 to between 60 and 65 mm Hg or the saturations from 90% to
92%. The PaO2 is limited to 60-65 mm Hg so as not to depress the patients respiratory drive. Studies show
that using oxygen at home for more than 15 hours a day increases quality of life and helps people live longer
when they have severe COPD and low blood levels of oxygen.
Generally, there are no risks from oxygen treatment at the recommended amount, but oxygen is a fire
hazard. Do not use oxygen around lit cigarettes, open flames, or anything flammable. Oxygen may worsen
the effects of paraquat poisoning and is not recommended for patients who have pulmonary fibrosis or other
lung damage caused by bleomycin.
13. Recognize the significance of clubbing.
Clubbing is a thickening of the flesh under the toenails and fingernails. The nail curves downward, similar to
the shape of the round part of an upside-down spoon. It is most often noted in heart and lung diseases that
cause a lower than normal amount of oxygen in the blood. Clubbing may also be due to lung cancer, and
diseases of the liver and gastrointestinal tract.
Clubbing is associated with:

Lung disease:
o Lung cancer, mainly large-cell lung cancer (35% of all cases), not seen frequently in small
cell lung cancer
o

Interstitial lung disease

Tuberculosis

Suppurative lung disease: lung abscess, empyema (=collection of pus within a naturally
existing anatomical cavity, such as the lung pleura), bronchiectasis, cystic fibrosis

Mesothelioma

CLUBBING IS NOT ASSOCIATED WITH COPD!!!

Heart disease:
o

Any disease featuring chronic hypoxia

Congenital cyanotic heart disease (most common cardiac cause)

Subacute bacterial endocarditis

Atrial myxoma (=benign tumor of mesenchymal heart cells)

Gastrointestinal and hepatobiliary:

o

Malabsorption

Crohn's disease and ulcerative colitis

Cirrhosis, especially in primary biliary cirrhosis

Hepatopulmonary syndrome (= dyspnea and hypoxemia caused by vasodilation in the lungs of

patients with liver disease. Dyspnea and hypoxemia are worse in the upright position, which is
called platypnea and orthodeoxia, respectively).

Others:
o

Hyperthyroidism (thyroid acropachy = subperiosteal new bone formation)

Familial and racial clubbing and "pseudoclubbing" (people of African descent often have what
appears to be clubbing)

14. Discuss the significance of the A-a gradient.

common measure of oxygenation. difference between the amount of the oxygen in the alveoli (ie, the
alveolar oxygen tension [PAO2]) and the amount of oxygen dissolved in the plasma (PaO2). PAO2 PaO2;
PAO2 = (FiO2 x [Patm - PH2O]) - (PaCO2 R) where FiO2 is the fraction of inspired oxygen (0.21 at room air),
Patm is the atmospheric pressure (760 mmHg at sea level), PH2O is the partial pressure of water (47 mmHg
at 37 degrees C), PaCO2 is the arterial carbon dioxide tension, and R is the respiratory quotient. The
respiratory quotient is approximately 0.8 at steady state, but varies according to the relative utilization of
carbohydrate, protein, and fat.
Estimate adequacy of oxygen transfer from the alveolus to pulmonary capillary blood. In the ideal alveolus,
every bit of oxygen inspired would rapidly cross over into the capillary blood, with an A-a gradient of 0. In
physiologic systems, with "normal" shunting of some blood, the A-a gradient can be up to 10 mmHg. In
certain pathologic conditions (diffusion impairment, V/Q mismatching, and shunt), the A-a gradient increases,
reflecting inadequacy of oxygen transfer. A gradient of 0-10 mmHg is considered normal; 10-20 is considered
mild impairment of oxygen exchange; 20-30 is considered moderate impairment; and > 30 is considered
severe gas exchange abnormality. Clinically, the A-a gradient can be used to assess gas exchange function
over time, and is thus useful in monitoring patients with certain types of respiratory distress.
15. List the mechanisms of hypoxia.
Hypoxia is decreased O2 delivery to the tissues. O2 delivery is a product of cardiac output and O2 content of
bllod, hypoxia is caused by decrease cardiac output (blood flow) or decreased O2 content of blood. O2
content of lbood is primarily determined by the amount of O2 hemoglobin.
Cause
Mechanism
- cardiac output
Blood flow
-Hypoxemia
PaO2
O2 sat of Hb
O2 content of blood
-Anemia
Hb concentration
O2 content of blood
-Carbon Monoxide poison
O2 content of blood
left shift of O2 Hb curve
Cyanide poison
O2 utilization by tissues
Some causes of hypoxemia are high altitude, hypoventilation, diffusion defect, V/Q defect, right to left shunt.
16. Describe pursed lip breathing and its physiological impact on ventialition.
Pursed lip breathing refers to puckering your lips. Breathing through pursed lips, the gas flow goes htrough
the little hole of the pucker. The small diameter of the hole causes high resistnace to airflow through it. This is

a method to keep airways open on forced exhale, it increases pressure along the arway. Airway collapse
occurs mainly in small airwasy that have little or no cartilage support. So a positive pressure inside will hold
htem open.
Normally the airway collapses on forced exhalation because the pleural pressure will be greater than the
airway pressure, but with pursed lip brathing, resistance is created which increases the pressure in the
airways upon exhalation, keeping the airway from collasing.
Pursed lip breathing is one of the simplest ways to control SOB. It provides a quick and easy way to slow
your pace of breathing, making each breath more effective.
Pursed lip breathing:

Improves ventilation
Releases trapped air in the lungs
Keeps the airways open longer and decreases the work of breathing
Prolongs exhalation to slow the breathing rate
Improves breathing patterns by moving old air out of the lungs and allowing for new air to enter the
lungs
Relieves SOB
Causes general relaxation