PANCE Blueprint Pulmonary (9%)

Emphysema

Patient with emphysema 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 sounds, pursed-lip breathing, end-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.

Spirometry is required to confirm COPD: a post-bronchodilator FEV1/FVC <0.70 establishes persistent airflow limitation that is not fully reversible (the key distinction from asthma).

GOLD grades severity by post-bronchodilator FEV1 (% predicted):

  • GOLD 1 (mild): FEV1 ≥80%
  • GOLD 2 (moderate): FEV1 50–79%
  • GOLD 3 (severe): FEV1 30–49%
  • GOLD 4 (very severe): FEV1 <30%

Supportive: a reduced DLCO reflects the alveolar destruction of emphysema.

Emphysema is a form of COPD due to structural changes in the lung - air spaces are enlarged as a consequence of the destruction of alveolar septae

  • Chronic bronchitis and emphysema almost always coexist
  • Chronic bronchitis (clinical features - a productive cough) vs. Emphysema (structural changes - enlarged airspaces)
    • Loss of elastin → Lungs are more compliant (lungs expand, hold air)
    • Alveolar air sacs permanently enlarge and lose elasticity → exhaling is difficult

Dyspnea and shortness of breath due to decreased gas exchange cause patients to exhale slowly through pursed lips to increase pressure in airways to keep airways from collapsing

  • Individuals are able to oxygenate blood (pink), but they have to purse their lips to do so (puffers) = Pink Puffers!
  • Alveoli are destroyed
  • Barrel chest due to air trapping and hyperinflation of lungs
  • Near-normal PaCO2 until late disease (they increase ventilation to blow off CO2); CO2 retention is typical of chronic bronchitis (blue bloaters)
  • Hyperinflation with bullae is a consistent finding in patients with emphysema
  • CXR reveals loss of lung markings and hyperinflation, tall lung fields, flat diaphragms
  • Normal hematocrit (HCT)

PATHOPHYSIOLOGY OF EMPHYSEMA - PROTEASE-ANTIPROTEASE IMBALANCE

"The underlying mechanism of emphysema involves a disruption of the protease-antiprotease balance. Chronic exposure to tobacco smoke recruits inflammatory cells (neutrophils and macrophages), which release proteolytic enzymes, specifically neutrophil elastase. Simultaneously, smoke inhibits alpha-1 antitrypsin, the enzyme responsible for neutralizing elastase. This imbalance leads to the destruction of elastin in the alveolar walls (septa), resulting in the permanent enlargement of airspaces, loss of elastic recoil, and air trapping."

CXR reveals loss of lung markings, hyperinflation, increased anterior-posterior diameter

  • PFTs show a decreased FEV1/FVC ratio + increased TLC (due to air trapping)
James Heilman, MD / CC BY-SA (https://creativecommons.org/licenses/by-sa/3.0)

Low, flat hemidiaphragm and loss of lung markings are consistent with emphysema. Image by James Heilman, MD, with labels by Stephen Pasquini PA-C, by CC 3.0

The most effective therapy for most patients with emphysema is smoking cessation

  • Treatment depends on severity: a combination of short-acting (SABA) or long-acting (LABA) beta 2 agonist and short-acting (SAMA) or long-acting (LAMA) muscarinic agent (also known as an anticholinergic agent) +/- inhaled glucocorticoids
  • GOLD assessment groups (A / B / E) guide initial therapy: Group B (more symptoms) and Group E (frequent/severe exacerbations) begin LABA + LAMA dual bronchodilator therapy; Group A begins a single bronchodilator. Add an inhaled corticosteroid (triple therapy) when blood eosinophils are ≥300 cells/µL (consider if ≥100 with ongoing exacerbations; avoid when <100). Roflumilast (chronic bronchitis with FEV1 <50% plus exacerbations), azithromycin, and dupilumab (type 2 inflammation) are add-on options for selected exacerbators
  • Long-term oxygen therapy in all patients with COPD who have chronic hypoxemia defined as (SpO2) ≤ 88%
  • Vaccination against both influenza and pneumococcal disease
  • COPD exacerbations are managed with systemic glucocorticoids, antibiotics (tailored to the likelihood of specific pathogens), and antiviral therapy when influenza is suspected
osmosis Osmosis
Picmonic
Emphysema

IM_MED_Emphysema_v1.5_

Emphysema is a form of chronic obstructive lung disease characterized by the 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.

Play Video + Quiz

Question 1
A 34-year-old man who has never smoked has 1 year of progressive exertional dyspnea. His father died of lung disease at age 45. Examination shows hyperresonance and decreased breath sounds at both lung bases. Spirometry shows a post-bronchodilator FEV1/FVC of 0.58. Chest CT shows emphysema that is most severe in the lower lobes. Which of the following is the most likely underlying cause?
A
Alpha-1 antitrypsin deficiency
B
Chronic asthma
Hint:
Asthma causes variable, largely reversible airflow obstruction; it does not cause lower-lobe panacinar emphysema in a young nonsmoker.
C
Cystic fibrosis
Hint:
Cystic fibrosis presents earlier with recurrent infections, upper-lobe bronchiectasis, and pancreatic insufficiency.
D
Idiopathic pulmonary fibrosis
Hint:
Idiopathic pulmonary fibrosis causes a restrictive pattern (normal or high FEV1/FVC) with basilar fibrosis in older adults.
E
Secondhand smoke exposure
Hint:
Smoke-related emphysema is centrilobular and upper-lobe predominant.
Question 1 Explanation: 
Alpha-1 antitrypsin deficiency is an inherited (codominant, SERPINA1 gene) cause of early-onset emphysema. Without enough alpha-1 antitrypsin, neutrophil elastase destroys alveolar walls, producing panacinar emphysema that is worst in the lower lobes, often before age 45 and earlier in smokers. It can also cause liver disease (cirrhosis). Cigarette smoking is still the most common cause of emphysema overall, but smoking causes centrilobular, upper-lobe disease. Every patient with COPD should be tested once with a serum alpha-1 antitrypsin level. Treatment is smoking cessation and standard COPD care, with IV augmentation therapy for severe deficiency.
Question 2
A 63-year-old woman with a 40 pack-year smoking history has 2 years of progressive dyspnea on exertion and a minimally productive morning cough. She is thin, with a barrel chest, hyperresonance to percussion, and diminished breath sounds. Which of the following is the most appropriate test to confirm the diagnosis?
A
Arterial blood gas
Hint:
An arterial blood gas measures oxygenation and CO2 retention to judge severity; it does not diagnose airflow obstruction.
B
Chest CT
Hint:
CT can show emphysema but is not needed to diagnose COPD and does not measure airflow.
C
Chest radiograph
Hint:
A chest radiograph may show hyperinflation and flattened diaphragms, but it cannot confirm or stage COPD.
D
Pulse oximetry
Hint:
Pulse oximetry screens for hypoxemia and guides oxygen therapy but does not diagnose COPD.
E
Spirometry with a bronchodilator
Question 2 Explanation: 
Spirometry is required to diagnose COPD: a post-bronchodilator FEV1/FVC below 0.70 confirms persistent airflow obstruction, and the FEV1 percent predicted grades severity (GOLD 1 to 4). In emphysema, lung volumes show air trapping (increased total lung capacity and residual volume) and the diffusing capacity (DLCO) is decreased from alveolar destruction, which helps separate emphysema from chronic bronchitis and asthma. Imaging and blood gases support the diagnosis and assess complications but do not replace spirometry.
Question 3
A 66-year-old man with emphysema (post-bronchodilator FEV1 52% predicted) becomes short of breath when walking at his own pace on level ground despite using albuterol as needed. He has had no exacerbations in the past year. His blood eosinophil count is 120 cells/µL. He quit smoking last year. Which of the following is the most appropriate maintenance therapy?
A
Daily oral prednisone
Hint:
Long-term oral corticosteroids have no role in stable COPD and cause major side effects.
B
Inhaled corticosteroid alone
Hint:
An inhaled corticosteroid is never used alone in COPD; it is added only for exacerbations with eosinophils of 300 or more and raises pneumonia risk.
C
Long-acting beta-agonist plus long-acting muscarinic antagonist
D
Oral theophylline
Hint:
Theophylline is a weak bronchodilator with a narrow therapeutic window and is not first-line.
E
Roflumilast
Hint:
Roflumilast is an add-on for frequent exacerbations in chronic bronchitis with FEV1 below 50%.
Question 3 Explanation: 
Per GOLD, this patient has significant symptoms (dyspnea when walking on level ground) and no recent exacerbations, which is group B. Initial maintenance therapy is a long-acting beta-agonist plus a long-acting muscarinic antagonist (LABA + LAMA), ideally in one inhaler (e.g., tiotropium-olodaterol or umeclidinium-vilanterol), with a short-acting bronchodilator for rescue. Group A (few symptoms) starts one long-acting bronchodilator; group E (exacerbations) starts LABA + LAMA and adds an inhaled corticosteroid only if blood eosinophils are 300 or more. Every patient also needs smoking cessation, vaccines, and pulmonary rehabilitation when symptomatic.
Question 4
A 70-year-old woman with COPD has 3 days of increased dyspnea, more sputum, and a change in sputum color to green. Temperature is 37.9°C (100.2°F), respirations are 22/min, and SpO2 is 89% on room air. She is alert and the chest radiograph shows no infiltrate. She has received nebulized albuterol and ipratropium. Which of the following is the most appropriate additional treatment?
A
High-flow oxygen to keep SpO2 at 100%
Hint:
Over-oxygenation worsens CO2 retention in COPD; the target SpO2 during an exacerbation is 88% to 92%.
B
Inhaled corticosteroid alone
Hint:
An inhaled corticosteroid alone does not treat an acute exacerbation and is never used as COPD monotherapy.
C
Intravenous aminophylline
Hint:
Methylxanthines such as aminophylline add side effects without benefit in exacerbations.
D
Observation without additional medication
Hint:
She has a moderate exacerbation with purulent sputum and needs systemic corticosteroids and an antibiotic, not observation.
E
Oral prednisone for 5 days plus an oral antibiotic
Question 4 Explanation: 
Treat a COPD exacerbation with the ABCs: Antibiotics, Bronchodilators, and Corticosteroids. Short-acting bronchodilators (albuterol with or without ipratropium) come first. A short course of systemic corticosteroids (prednisone 40 mg daily for 5 days) speeds recovery and lowers the chance of relapse. Antibiotics (such as amoxicillin-clavulanate, doxycycline, or azithromycin) are indicated when at least two of the three cardinal symptoms are present (more dyspnea, more sputum, purulent sputum), or when ventilation is needed. Give controlled oxygen to a target SpO2 of 88% to 92%, and use noninvasive ventilation for acute hypercapnic respiratory acidosis (pH below 7.35).
Question 5
A 68-year-old man with severe emphysema quit smoking 2 years ago and uses a LABA-LAMA inhaler daily. At a stable follow-up visit, resting room-air pulse oximetry is 86% and an arterial blood gas shows a PaO2 of 52 mmHg. Which of the following is most likely to improve his survival?
A
Chronic azithromycin
Hint:
Chronic azithromycin can reduce exacerbations in selected patients but has not been shown to prolong survival.
B
Daily inhaled corticosteroid
Hint:
Inhaled corticosteroids may reduce exacerbations in eosinophilic COPD but do not reliably improve survival and raise pneumonia risk.
C
Long-term oxygen therapy
D
Oral theophylline
Hint:
Theophylline offers modest bronchodilation and no survival benefit.
E
Pulmonary rehabilitation
Hint:
Pulmonary rehabilitation improves dyspnea, exercise capacity, and quality of life, but long-term oxygen is the therapy proven to lower mortality in chronic hypoxemia.
Question 5 Explanation: 
In COPD, smoking cessation and long-term oxygen therapy are the interventions proven to reduce mortality. Long-term oxygen (at least 15 hours a day) is indicated for chronic resting hypoxemia: PaO2 of 55 mmHg or less or SpO2 of 88% or less, or PaO2 56 to 59 mmHg with cor pulmonale, edema, or polycythemia. Recheck oxygenation after 60 to 90 days on therapy. Lung volume reduction (surgical or endobronchial valves) can also help selected patients with upper-lobe emphysema.
Question 6
A 62-year-old man with emphysema comes for a routine visit. He quit smoking 6 months ago and uses a long-acting bronchodilator. Resting pulse oximetry is 93% on room air, and he has had no exacerbations this year. Which of the following is the most appropriate additional intervention at this time?
A
Annual influenza vaccination
B
Daily prophylactic amoxicillin
Hint:
Prophylactic antibiotics are not recommended for stable COPD; chronic azithromycin is reserved for selected patients with frequent exacerbations.
C
Home oxygen therapy
Hint:
Home oxygen is indicated only for resting hypoxemia (SpO2 88% or less, or PaO2 55 mmHg or less); his saturation is 93%.
D
Inhaled corticosteroid monotherapy
Hint:
An inhaled corticosteroid is never used alone in COPD.
E
Maintenance oral prednisone
Hint:
Maintenance oral corticosteroids are not recommended for stable COPD.
Question 6 Explanation: 
Every patient with COPD should be vaccinated to prevent exacerbations and pneumonia: annual influenza vaccine, pneumococcal vaccine, COVID-19 vaccine, RSV vaccine (adults 75 and older, and adults 60 to 74 with COPD or another risk condition), Tdap if never received as an adult, and recombinant zoster vaccine at 50 and older. COPD is an indication for pneumococcal vaccination at any adult age. Influenza vaccination lowers serious illness, hospitalization, and death in COPD. Smoking cessation and pulmonary rehabilitation are the other cornerstones of care for all patients.
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References: Merck Manual · UpToDate

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