PANCE Blueprint Pulmonary (12%)

Emphysema

Patient will present as → a 65 year-old male complaining of fatigue and shortness of breath with exertion. The patient reports minimal cough. On physical exam you note a thin, barrel chested man with decreased heart and breath soundspursed-lip breathingend-expiratory wheezing, and scattered rhonchi. Chest X-ray reveals a flattened diaphragm, hyperinflation and a small, thin appearing heart. PFTs show a decreased FEV1 / FVC ratio.

Emphysema is a condition in which air spaces are enlarged as a consequence of destruction of alveolar septae.

The body's natural response to ↓ lung function is chronic hyperventilation = Pink Puffers!

  • Alveoli are destroyed
  • CO2 Retainers - the body must increase ventilation to blow off CO2
  • Hyperinflation with bullae is a consistent finding in patients with emphysema
  • CXR = Tall lung fields, flat diaphragms, Barrel chest
  • Normal hematocrit (HCT)

CXR reveals loss of lung markings and hyperinflation

  • Parenchymal bullae and blebs are pathognomonic

Management is the same as Chronic Bronchitis

  • Ipratropium (also albuterol inhaler)
  • O2
  • Oral steroids
  • Antibiotics (see Chronic bronchitis)
Emphysema

Low, flat hemidaphragm and loss of lung markings is consistent with emphysema

IM_MED_Emphysema_v1.5_ Emphysema is a form of chronic obstructive lung disease characterized by destruction of alveolar walls. There are two main forms of emphysema. Centriacinar emphysema is the most common form and associated with many years of smoking. Panacinar emphysema is less common and associated with individuals with an alpha-1 antitrypsin deficiency. Overall, emphysema is characterized by increased elastase activity, increased lung compliance, enlargement of air spaces and decreased recoil of the lungs. Individuals with emphysema are commonly called pink puffers and have increased anteroposterior diameter of the chest, often referred to as a barrel chest. They also commonly exhale through pursed lips which help keep their airways open during exhalation.

Emphysema Picmonic

Question 1
A 62 year-old male presents with a history of dyspnea on exertion and chronic cough worse with arising in the mornings. He has a 40-year-pack history of cigarette use. On examination there is increased AP diameter and decreased breath sounds with a prolonged expiratory phase. Pulse oximetry reveals an oxygen saturation of 93% on room air. In addition to smoking cessation, which of the following is an appropriate intervention at this time?
A
Home oxygen therapy
Hint:
Home oxygen therapy is indicated in COPD patients with an oxygen saturation < or equal to 88% or a pO2 < or equal to 55 mm Hg taken at rest breathing room air.
B
Maintenance oral steroids
Hint:
While oral steroids may be utilized in treatment of COPD, they are usually reserved for end stage disease due to the multiple systemic side effects of prolonged use. In addition only about 10% of patients show any increase in FEV1 and there use should be reserved for patients who show a 20% or greater improvement in FEV1.
C
Prophylactic antibiotic therapy
Hint:
Use of antibiotics should be reserved for treatment of acute exacerbations of COPD, acute bronchitis or documented bacterial infections, not prophylaxis.
D
Recommend influenza and pneumococcal vaccines
Question 1 Explanation: 
In addition to smoking cessation, patients may benefit from vaccination against both influenza and pneumococcal disease.
Question 2
Which of the following best describes the pathophysiology of emphysema?
A
Interstitial inflammation and fibrosis
Hint:
Interstitial inflammation and fibrosis are seen with restrictive causes of lung disease, such as asbestosis.
B
Alveolar enlargement and loss of septa
C
Mucosal edema and inflammatory response
Hint:
Mucosal edema and inflammatory response are seen with asthma.
D
Excessive mucus secretion and chronic cough
Hint:
Excessive mucus secretion and chronic cough are characteristic of chronic bronchitis.
Question 2 Explanation: 
Emphysema results from alveolar enlargement with loss of septal wall integrity without any evidence of fibrosis.
Question 3
Which of the following physical exam findings is consistent with moderate emphysema?
A
Increased tactile fremitus
Hint:
Physical examination findings in emphysema include a midline trachea, diffuse hyperresonant to percussion, and decreased tactile fremitus.
B
Dullness to percussion
Hint:
See A for explanation.
C
Distant heart sounds
D
Deviated trachea
Hint:
See A for explanation.
Question 3 Explanation: 
Distant heart sounds are common in emphysema patients due to hyperinflation of the lungs.
Question 4
A 60 year-old patient with COPD characteristic of emphysema presents with a cough and increased sputum production. The following information is noted: Temperature 100°F (37.8°C); Respiratory rate 20/min; Heart rate 88 beats/min; pH 7.44; PaO2 75 mmHg; PaCO2 40 mmHg; O2 saturation 92%. Physical examination is remarkable for increased AP diameter, diminished breath sounds without wheezes, rhonchi, or other signs of respiratory distress. Which of the following would be an appropriate treatment for this patient?
A
Broad-spectrum antibiotic
B
Admission to the hospital
Hint:
Admission is only warranted if the patient's respiratory status requires ventilatory assistance. This patient's blood gases are unremarkable for a patient with COPD and the patient is not in respiratory distress.
C
Oxygen at 6 L/min by nasal cannula
Hint:
Oxygen therapy should only be used for severe hypoxemia and should only be given at a low concentration, such as 2 L/min. Higher dose oxygen may stop the hypoxemic ventilatory drive.
D
Brief course of oral theophylline
Hint:
Oral theophylline is considered a secondary bronchodilator. The use of a metered-dose inhaler would be a preferable first-line treatment if this method of treatment were chosen.
Question 4 Explanation: 
Sputum production is extremely variable from patient to patient, but any increase in sputum with a history of COPD reported by a patient must be regarded as potentially infectious and treated promptly.
Question 5
A 24 year-old male presents complaining of a 9 month history of increasing shortness of breath, dyspnea on exertion, and a cough productive of white sputum, mostly in the mornings. He denies orthopnea, PND, peripheral edema, fever, chills, night sweats, recent changes in weight, palpitations, chest pain, food intolerances, or other complaints. Patient has a history of recurrent lung infections. He states that his father had chronic pulmonary problems and died at age 42 from unknown lung disease. The patient denies smoking, alcohol or illicit drug use. On physical examination, the respiratory rate is 22 per minute, pulse of 98 bpm, temperature of 98.7 degrees. Pulmonary exam reveals end-expiratory wheezes bilaterally and hyperresonance to percussion. His cardiac exam is normal. Chest x- ray shows decreased lung markings. ECG is normal. Pulmonary function tests show an FEV1 63% of expected and residual capacity is 123% of expected. Which of the following is the most likely diagnosis?
A
Emphysema
B
Pulmonary fibrosis
Hint:
The PFTs from a person with pulmonary fibrosis would be consistent with a restrictive pattern. This patient has an obstructive pattern of lung disease.
C
Ventricular septal defect
Hint:
Ventricular septal defect will have a systolic murmur associated with it.
D
Congestive heart failure
Hint:
Congestive heart failure might explain some of the symptoms of this patient (increasing shortness of breath and DOE), he denies other common symptoms, such as orthopnea and peripheral edema. CHF should not result in changes in the PFTs.
Question 5 Explanation: 
This person has an obstructive lung disease based on PFTs. Emphysema is the most likely diagnosis, and may be related to alpha-1 antitrypsin deficiency based on family history and lack of smoking history and young age.
Question 6
A 56 year-old female with a 35 pack year smoking history presents to the clinic with shortness of breath and cough. On examination, she is thin with no recent weight loss. She appears uncomfortable, breath sounds are diminished without adventitious sounds. Pulmonary function tests show a marked increase in total lung capacity (TLC) and a decreased FEV1. What is the most likely diagnosis for this patient?
A
Persistent asthma
Hint:
Lung function in asthma is evaluated by FEV1/FVC ratio with reduction noted with airflow obstruction.
B
Chronic obstructive pulmonary disease
C
Idiopathic fibrosing interstitial pneumonia
Hint:
Pulmonary function testing in idiopathic fibrosing interstitial pneumonia shows a loss of lung volume with normal to increased airflow rates in interstitial lung disease.
D
Sarcoidosis
Hint:
Restrictive changes with decreased lung volumes and diffusing capacity are common in sarcoidosis.
Question 6 Explanation: 
Lung volume measurements in COPD reveal a marked increase in residual volume indicative of air trapping.
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