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ABIM Pulmonary practice questions

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Question 1Obstructive Lung Disease

Which spirometric finding establishes the diagnosis of chronic obstructive pulmonary disease (COPD) in a symptomatic adult?

  1. Post-bronchodilator FEV1/FVC ratio below 0.70 Correct answer.
  2. FEV1 less than 80% of predicted
  3. Pre-bronchodilator FEV1/FVC ratio below 0.70
  4. Reduced diffusing capacity for carbon monoxide

Explanation

COPD requires persistent airflow limitation, defined after bronchodilator administration as an FEV1/FVC ratio below 0.70. Pre-bronchodilator values alone can reflect reversible obstruction and are insufficient. FEV1 percent predicted grades severity but does not establish the diagnosis, and diffusing capacity is not diagnostic.

Question 2Obstructive Lung Disease

Which post-bronchodilator response on spirometry supports a diagnosis of asthma?

  1. A peak expiratory flow variability of 8% over one week
  2. An increase in FVC of 200 mL and 10% from baseline
  3. An increase in FEV1 of 250 mL and 15% from baseline Correct answer.
  4. An increase in FEV1 of 100 mL and 8% from baseline

Explanation

Asthma is supported by an increase in FEV1 of at least 12% and 200 mL after a short-acting bronchodilator. Smaller changes can occur in COPD or with normal test variation. Peak flow variability above 10% also supports asthma, but 8% does not meet that threshold.

Question 3Obstructive Lung Disease

Which medication acts as a partial agonist at nicotinic acetylcholine receptors and is used for smoking cessation?

  1. Bupropion
  2. Clonidine
  3. Nortriptyline
  4. Varenicline Correct answer.

Explanation

Varenicline partially stimulates nicotinic acetylcholine receptors, which reduces craving and withdrawal while blocking nicotine reward. Bupropion inhibits dopamine and norepinephrine reuptake. Nortriptyline and clonidine are second-line agents with different mechanisms.

Question 4Obstructive Lung Disease

A 38-year-old nonsmoker has disproportionate emphysema on CT and a low serum alpha-1 antitrypsin level. Which pattern is most characteristic?

  1. Diffuse interstitial fibrosis with honeycombing
  2. Apical bullae with preserved lower lobes
  3. Panacinar emphysema with lower-lobe predominance Correct answer.
  4. Centriacinar emphysema predominantly in the upper lobes

Explanation

Alpha-1 antitrypsin deficiency causes panacinar emphysema that is usually more prominent in the lower lobes and may present at a young age in nonsmokers. Smoking-related emphysema is typically centriacinar and upper-lobe predominant. Interstitial fibrosis is not the characteristic pattern.

Question 5Obstructive Lung Disease

Which patient with an acute COPD exacerbation is most likely to benefit from antibiotic therapy?

  1. A patient with isolated fatigue and a normal white blood cell count
  2. A patient with stable sputum volume and new ankle edema
  3. A patient with wheezing and a clear chest radiograph
  4. A patient with increased sputum purulence and increased dyspnea Correct answer.

Explanation

Antibiotics are indicated when increased sputum purulence occurs together with increased dyspnea or increased sputum volume, corresponding to Anthonisen criteria. Purulence suggests a bacterial cause. Wheezing alone, edema, or fatigue without other features does not establish a bacterial exacerbation.

Question 6Obstructive Lung Disease

Which patient with COPD is most likely to benefit from adding an inhaled corticosteroid to long-acting bronchodilator therapy?

  1. A patient with chronic productive cough, frequent wheezing, and a blood eosinophil count of 100 cells/µL
  2. A patient with two exacerbations in the past year and a blood eosinophil count of 350 cells/µL Correct answer.
  3. A patient with mild dyspnea and a history of pneumonia after prior ICS use
  4. A patient with FEV1 65% predicted and no exacerbations in the past year

Explanation

Inhaled corticosteroids are most useful in COPD patients with recurrent exacerbations, particularly when blood eosinophils are elevated, such as 300 cells/µL or higher. They are not routinely indicated for mild disease without exacerbations. A history of pneumonia is a reason for caution.

Question 7Obstructive Lung Disease

What is the preferred long-term controller medication for persistent asthma?

  1. A long-acting beta-2 agonist used alone
  2. A short-acting beta-2 agonist used before exercise
  3. A leukotriene receptor antagonist used alone
  4. An inhaled corticosteroid Correct answer.

Explanation

Inhaled corticosteroids are the most effective anti-inflammatory controller and form the foundation of persistent asthma treatment. Long-acting beta-2 agonist monotherapy is unsafe because it does not treat underlying inflammation. Leukotriene antagonists are alternatives but are less effective as monotherapy.

Question 8Obstructive Lung Disease

A patient with a severe asthma exacerbation remains distressed after repeated inhaled albuterol and systemic corticosteroids. Which adjunctive therapy is supported for severe exacerbations?

  1. Oral montelukast
  2. Inhaled cromolyn sodium
  3. Intravenous aminophylline
  4. Intravenous magnesium sulfate Correct answer.

Explanation

Intravenous magnesium sulfate can be added for severe asthma exacerbations that respond poorly to initial bronchodilators and systemic steroids. Montelukast and cromolyn have no role in acute severe exacerbation. Aminophylline is not recommended because of toxicity and limited benefit.

Question 9Obstructive Lung Disease

Which biologic directly targets the interleukin-5 pathway in severe eosinophilic asthma?

  1. Omalizumab
  2. Mepolizumab Correct answer.
  3. Dupilumab
  4. Tezepelumab

Explanation

Mepolizumab is an anti-IL-5 monoclonal antibody that reduces eosinophilic inflammation and exacerbations. Omalizumab binds IgE, dupilumab blocks the IL-4 receptor alpha, and tezepelumab targets thymic stromal lymphopoietin.

Question 10Obstructive Lung Disease

Which imaging study is most appropriate to confirm suspected bronchiectasis?

  1. Ventilation-perfusion lung scan with quantitative analysis
  2. High-resolution computed tomography Correct answer.
  3. Chest radiograph with lateral view
  4. Flexible bronchoscopy with bronchoalveolar lavage

Explanation

High-resolution computed tomography is the test of choice because it shows airway dilation, wall thickening, and lack of tapering with high sensitivity. Chest radiography is insensitive and may be normal. Ventilation-perfusion scanning and bronchoscopy do not establish the diagnosis.

Question 11Obstructive Lung Disease

Which test is the standard confirmatory test for cystic fibrosis?

  1. Sweat chloride concentration Correct answer.
  2. Serum immunoreactive trypsinogen
  3. Nasal potential difference
  4. Sputum bacterial culture

Explanation

Sweat chloride testing by quantitative pilocarpine iontophoresis is the standard confirmatory test; a value of 60 mmol/L or higher is diagnostic. Immunoreactive trypsinogen is a newborn screening test, not confirmatory. Nasal potential difference and genetic testing are adjuncts in equivocal cases.

Question 12Obstructive Lung Disease

Which intervention is a cornerstone of daily management for patients with bronchiectasis and chronic sputum production?

  1. Airway clearance techniques Correct answer.
  2. Routine long-term oral corticosteroids
  3. Prophylactic oral antibiotics for all patients
  4. Continuous inhaled short-acting bronchodilators

Explanation

Regular airway clearance, such as chest physiotherapy and oscillating positive expiratory pressure devices, helps mobilize secretions and reduce exacerbations. Long-term oral steroids are not routine. Prophylactic antibiotics are reserved for selected patients with frequent exacerbations.

Question 13Obstructive Lung Disease

Which finding qualifies a patient with COPD for long-term home oxygen therapy?

  1. SpO2 of 90% on room air with dyspnea on exertion
  2. FEV1 of 30% predicted with normal arterial oxygen tension
  3. PaO2 of 58 mm Hg without cor pulmonale or polycythemia
  4. PaO2 of 54 mm Hg while breathing room air at rest Correct answer.

Explanation

Long-term oxygen therapy is indicated when PaO2 is 55 mm Hg or lower or SpO2 is 88% or lower at rest. A PaO2 of 56 to 59 mm Hg qualifies only with evidence of end-organ effects such as cor pulmonale or polycythemia. FEV1 alone is not an indication.

Question 14Obstructive Lung Disease

Which patient with severe COPD is the best candidate for lung volume reduction surgery?

  1. A patient with upper-lobe–predominant emphysema and low baseline exercise capacity Correct answer.
  2. A patient with lower-lobe emphysema and a DLCO of 15% predicted
  3. A patient with homogeneous emphysema, an FEV1 of 20% predicted, and continued smoking
  4. A patient who continues to smoke and has an FEV1 of 30% predicted

Explanation

The NETT trial showed survival and functional benefit mainly in patients with upper-lobe–predominant emphysema and low baseline exercise capacity. Homogeneous disease and very low DLCO carry higher surgical risk. Active smoking is a contraindication.

Question 15Obstructive Lung Disease

A woman with persistent asthma becomes pregnant. How should her inhaled corticosteroid controller be managed?

  1. Continue the inhaled corticosteroid Correct answer.
  2. Replace it with a long-acting beta-2 agonist alone
  3. Stop it during the first trimester
  4. Switch to daily oral prednisone

Explanation

Inhaled corticosteroids should be continued in pregnancy because uncontrolled asthma increases the risk of preterm birth and low birth weight. Long-acting beta-2 agonist monotherapy is inappropriate. Oral steroids are reserved for severe exacerbations.

Question 16Obstructive Lung Disease

A lung transplant recipient develops progressive airflow obstruction months after transplantation. What is the most likely diagnosis?

  1. Bronchiolitis obliterans syndrome Correct answer.
  2. Cytomegalovirus pneumonitis with bilateral infiltrates
  3. Anastomotic airway stenosis
  4. Acute cellular rejection

Explanation

Bronchiolitis obliterans syndrome is the clinical manifestation of chronic lung allograft dysfunction and presents with progressive FEV1 decline and airflow obstruction. Acute cellular rejection usually occurs earlier and is diagnosed histologically. Anastomotic stenosis causes focal obstruction, and CMV pneumonitis causes infiltrates rather than progressive obstruction.

Question 17Obstructive Lung Disease

Which intervention most consistently improves dyspnea and quality of life in symptomatic COPD?

  1. Pulmonary rehabilitation Correct answer.
  2. Long-term oral corticosteroids
  3. Continuous supplemental oxygen for patients with normal oxygen saturation
  4. Routine antibiotics every month

Explanation

Pulmonary rehabilitation combines exercise training and education and improves dyspnea, exercise capacity, and quality of life. Long-term oral steroids cause harm and are not recommended. Oxygen does not improve outcomes when oxygen saturation is normal.

Question 18Obstructive Lung Disease

A patient with COPD has an mMRC score of 2, one moderate exacerbation in the past year, and an FEV1 of 55% predicted. Which GOLD group does this represent?

  1. Group E
  2. Group C
  3. Group B Correct answer.
  4. Group A

Explanation

Group B includes patients with 0 to 1 moderate exacerbations and greater symptoms, defined as an mMRC score of 2 or higher or a CAT score of 20 or higher. Group E requires at least two moderate exacerbations or one hospitalization. Group A has fewer symptoms with low exacerbation risk.

Question 19Obstructive Lung Disease

A patient with a COPD exacerbation has a pH of 7.25, PaCO2 of 68 mm Hg, and respiratory distress despite bronchodilators and steroids. What is the next best management?

  1. Immediate endotracheal intubation and mechanical ventilation
  2. Intravenous theophylline
  3. Noninvasive positive pressure ventilation Correct answer.
  4. High-flow nasal oxygen alone with close monitoring

Explanation

Noninvasive positive pressure ventilation is the preferred ventilatory support for hypercapnic respiratory acidosis from a COPD exacerbation when the patient can protect the airway. It reduces the need for intubation and lowers mortality. Intubation is reserved for failure of noninvasive ventilation or contraindications.

Question 20Obstructive Lung Disease

A patient has episodic wheeze and cough but normal spirometry. Which test is most useful to evaluate for bronchial hyperresponsiveness suggesting asthma?

  1. Chest computed tomography
  2. Methacholine challenge Correct answer.
  3. Sputum culture
  4. Serum IgE level

Explanation

A methacholine challenge can demonstrate bronchial hyperresponsiveness when spirometry is normal and the diagnosis remains uncertain. A negative challenge makes current asthma unlikely. Chest CT and sputum culture evaluate structural or infectious causes, and IgE is not diagnostic.

Question 21Diffuse Parenchymal/Interstitial Lung Disease

A 67-year-old man has progressive exertional dyspnea, bibasilar velcro crackles, and clubbing. High-resolution CT shows subpleural, basal-predominant honeycombing with traction bronchiectasis, and no alternative cause is identified. What is the most likely diagnosis?

  1. Cryptogenic organizing pneumonia
  2. Idiopathic pulmonary fibrosis Correct answer.
  3. Hypersensitivity pneumonitis
  4. Pulmonary sarcoidosis

Explanation

A usual interstitial pneumonia pattern on high-resolution CT in the appropriate clinical setting supports idiopathic pulmonary fibrosis after known causes are excluded. Honeycombing with basal and subpleural predominance is the radiologic hallmark. This pattern is not typical of hypersensitivity pneumonitis, sarcoidosis, or organizing pneumonia.

Question 22Diffuse Parenchymal/Interstitial Lung Disease

Which high-resolution CT finding is most characteristic of usual interstitial pneumonia?

  1. Bilateral hilar adenopathy
  2. Migratory patchy consolidation
  3. Subpleural basal honeycombing Correct answer.
  4. Upper-lobe predominant centrilobular nodules

Explanation

Usual interstitial pneumonia is characterized by subpleural and basal-predominant reticulation, honeycombing, and traction bronchiectasis. These features reflect progressive fibrotic remodeling. Centrilobular nodules, migratory consolidation, and hilar adenopathy suggest other interstitial or granulomatous diseases.

Question 23Diffuse Parenchymal/Interstitial Lung Disease

A 52-year-old woman who keeps pigeons has insidious dyspnea. High-resolution CT shows diffuse centrilobular nodules and air trapping, and serum precipitins to avian antigens are positive. What is the most likely diagnosis?

  1. Acute eosinophilic pneumonia with peripheral eosinophilia
  2. Idiopathic pulmonary fibrosis with usual interstitial pneumonia
  3. Chronic hypersensitivity pneumonitis Correct answer.
  4. Pulmonary sarcoidosis with bilateral hilar adenopathy

Explanation

Chronic exposure to an inhaled antigen with centrilobular nodules, air trapping, and positive precipitins supports chronic hypersensitivity pneumonitis. Antigen avoidance and anti-inflammatory therapy are central management considerations. The radiologic pattern is not usual interstitial pneumonia, sarcoidosis, or eosinophilic pneumonia.

Question 24Diffuse Parenchymal/Interstitial Lung Disease

A 34-year-old African American man has asymptomatic bilateral hilar lymphadenopathy and erythema nodosum. What is the most likely diagnosis?

  1. Tuberculosis
  2. Sarcoidosis Correct answer.
  3. Lymphoma
  4. Silicosis

Explanation

Bilateral hilar adenopathy with erythema nodosum in a young adult is characteristic of Löfgren syndrome, a form of sarcoidosis. It often has a favorable prognosis. Silicosis, tuberculosis, and lymphoma can cause adenopathy but do not fit this classic syndromic presentation as well.

Question 25Diffuse Parenchymal/Interstitial Lung Disease

A 70-year-old retired shipyard worker has pleural plaques and bibasilar reticular opacities. Which exposure is most likely responsible?

  1. Beryllium
  2. Coal dust
  3. Asbestos Correct answer.
  4. Silica

Explanation

Asbestos exposure causes pleural plaques and can lead to asbestosis, a diffuse interstitial fibrosis that is usually basal predominant. The combination of pleural plaques and lower-lobe reticulation is highly suggestive. Silica, beryllium, and coal dust produce different radiographic patterns and exposure histories.

Question 26Diffuse Parenchymal/Interstitial Lung Disease

A construction worker who performed sandblasting has upper-lobe lung nodules and eggshell calcification of the hilar lymph nodes. What is the most likely diagnosis?

  1. Silicosis Correct answer.
  2. Hypersensitivity pneumonitis
  3. Asbestosis
  4. Sarcoidosis

Explanation

Silicosis classically produces upper-lobe nodules and eggshell hilar lymph node calcification in a person with silica exposure. Sandblasting is a well-recognized high-risk exposure. Asbestosis, sarcoidosis, and hypersensitivity pneumonitis have different radiologic patterns and exposure associations.

Question 27Diffuse Parenchymal/Interstitial Lung Disease

A 48-year-old woman with scleroderma has bilateral ground-glass opacities with fine reticulation and lower-lobe predominance on high-resolution CT. Surgical biopsy shows uniform interstitial inflammation and fibrosis without honeycombing. Which pattern is most likely?

  1. Cryptogenic organizing pneumonia with consolidation
  2. Nonspecific interstitial pneumonia Correct answer.
  3. Diffuse alveolar damage with hyaline membranes
  4. Usual interstitial pneumonia with honeycombing

Explanation

Nonspecific interstitial pneumonia shows temporally uniform interstitial inflammation and fibrosis and is often associated with connective tissue disease. Ground-glass opacity with fine reticulation and lower-lobe predominance is typical. Usual interstitial pneumonia shows temporal heterogeneity and honeycombing, while organizing pneumonia and diffuse alveolar damage have different histologic features.

Question 28Diffuse Parenchymal/Interstitial Lung Disease

A patient with mechanic's hands, proximal muscle weakness, and interstitial lung disease is evaluated. Which autoantibody is most strongly associated with this presentation?

  1. Anti-Scl-70
  2. Anti-CCP
  3. Anti-centromere
  4. Anti-Jo-1 Correct answer.

Explanation

Anti-Jo-1 is the most common antisynthetase antibody and is associated with antisynthetase syndrome, which includes myositis, mechanic's hands, arthritis, and interstitial lung disease. Recognition is important because ILD can be the dominant manifestation. Anti-centromere, anti-Scl-70, and anti-CCP are associated with other connective tissue diseases.

Question 29Diffuse Parenchymal/Interstitial Lung Disease

Which medication is most strongly associated with cumulative dose-related pulmonary fibrosis and requires monitoring of pulmonary function during therapy?

  1. Acetaminophen
  2. Omeprazole
  3. Albuterol
  4. Bleomycin Correct answer.

Explanation

Bleomycin can cause dose-related pneumonitis and pulmonary fibrosis, and cumulative dose is an important risk factor. Pulmonary function monitoring is used during therapy. Albuterol, omeprazole, and acetaminophen are not associated with this pattern of drug-induced fibrosis.

Question 30Diffuse Parenchymal/Interstitial Lung Disease

A 42-year-old man has progressive dyspnea, and high-resolution CT shows bilateral ground-glass opacities with smooth septal thickening in a crazy-paving pattern. BAL returns milky fluid, and biopsy shows PAS-positive lipoproteinaceous material. What is the most likely diagnosis?

  1. Lymphangioleiomyomatosis with renal angiomyolipoma
  2. Chronic eosinophilic pneumonia with peripheral consolidation
  3. Pulmonary alveolar proteinosis Correct answer.
  4. Pulmonary sarcoidosis with hilar adenopathy

Explanation

Pulmonary alveolar proteinosis classically causes crazy-paving on CT and BAL fluid that is milky or opaque because of surfactant-derived lipoproteinaceous material. PAS-positive material within alveolar spaces supports the diagnosis. The other options do not produce this characteristic BAL and imaging pattern.

Question 31Diffuse Parenchymal/Interstitial Lung Disease

A 35-year-old woman with tuberous sclerosis has recurrent pneumothorax, diffuse thin-walled lung cysts, and a chylothorax. What is the most likely diagnosis?

  1. Idiopathic pulmonary fibrosis
  2. Lymphangioleiomyomatosis Correct answer.
  3. Pulmonary Langerhans cell histiocytosis
  4. Birt-Hogg-Dubé syndrome

Explanation

Lymphangioleiomyomatosis causes diffuse thin-walled cysts, recurrent pneumothorax, and chylous pleural effusions, and it is strongly associated with tuberous sclerosis. The cysts are typically uniform and diffuse. Idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, and Birt-Hogg-Dubé syndrome have different imaging distributions and associations.

Question 32Diffuse Parenchymal/Interstitial Lung Disease

A 28-year-old male smoker has recurrent pneumothorax and high-resolution CT showing upper-lobe cysts and nodules. Biopsy reveals Langerhans cells. What is the most likely diagnosis?

  1. Pulmonary Langerhans cell histiocytosis Correct answer.
  2. Lymphangioleiomyomatosis with chylothorax
  3. Sarcoidosis with perilymphatic nodules
  4. Chronic hypersensitivity pneumonitis with air trapping

Explanation

Pulmonary Langerhans cell histiocytosis occurs predominantly in smokers and produces upper-lobe cysts and nodules, often with recurrent pneumothorax. Identification of Langerhans cells supports the diagnosis. Sarcoidosis, hypersensitivity pneumonitis, and lymphangioleiomyomatosis have different radiologic patterns and risk factors.

Question 33Diffuse Parenchymal/Interstitial Lung Disease

A 38-year-old woman has acute fever, dyspnea, and bilateral peripheral ground-glass and consolidative opacities. BAL shows 45% eosinophils, and infection is excluded. What is the most likely diagnosis?

  1. Hypersensitivity pneumonitis
  2. Granulomatosis with polyangiitis
  3. Cryptogenic organizing pneumonia
  4. Acute eosinophilic pneumonia Correct answer.

Explanation

Acute eosinophilic pneumonia presents with acute febrile respiratory failure, diffuse or peripheral opacities, and marked BAL eosinophilia. Infection and other causes of eosinophilia must be excluded. Organizing pneumonia, hypersensitivity pneumonitis, and granulomatosis with polyangiitis do not typically cause such prominent BAL eosinophilia.

Question 34Diffuse Parenchymal/Interstitial Lung Disease

An aerospace worker develops granulomatous interstitial lung disease, and the beryllium lymphocyte proliferation test is positive. What is the most likely diagnosis?

  1. Silicosis with eggshell hilar calcification
  2. Chronic beryllium disease Correct answer.
  3. Sarcoidosis with bilateral hilar adenopathy
  4. Asbestosis with pleural plaques

Explanation

Chronic beryllium disease is a granulomatous lung disease caused by beryllium sensitization, confirmed by a positive beryllium lymphocyte proliferation test. It can closely mimic sarcoidosis. The occupational exposure and positive sensitization test distinguish it from the other options.

Question 35Diffuse Parenchymal/Interstitial Lung Disease

A 45-year-old woman has hemoptysis, anemia, and diffuse alveolar opacities. Serology is positive for anti-glomerular basement membrane antibodies. What is the most likely diagnosis?

  1. Idiopathic pulmonary fibrosis
  2. Pulmonary sarcoidosis
  3. Pulmonary alveolar proteinosis
  4. Goodpasture syndrome Correct answer.

Explanation

Anti-glomerular basement membrane disease involving the lungs and kidneys is Goodpasture syndrome. Diffuse alveolar hemorrhage causes hemoptysis, anemia, and alveolar opacities. The positive anti-GBM antibody establishes the diagnosis.

Question 36Diffuse Parenchymal/Interstitial Lung Disease

In idiopathic pulmonary fibrosis, which variable is most consistently associated with increased mortality during follow-up?

  1. Decline in forced vital capacity Correct answer.
  2. High diffusing capacity for carbon monoxide
  3. FEV1/FVC ratio greater than 0.8
  4. Absence of digital clubbing

Explanation

Serial decline in forced vital capacity is a well-established predictor of mortality in idiopathic pulmonary fibrosis and is used to assess disease progression. A low diffusing capacity also carries a poor prognosis, but a high value does not. Clubbing and spirometric ratio are not the main mortality markers.

Question 37Diffuse Parenchymal/Interstitial Lung Disease

Which histopathologic feature most strongly favors fibrotic hypersensitivity pneumonitis over idiopathic pulmonary fibrosis?

  1. Subpleural honeycombing with temporal heterogeneity
  2. Fibroblast foci with patchy lung involvement
  3. Bronchiolocentric granulomatous fibrosis Correct answer.
  4. Smooth muscle hyperplasia in small airways

Explanation

Fibrotic hypersensitivity pneumonitis often shows bronchiolocentric inflammation and poorly formed granulomas in addition to fibrosis. These features point to an antigen-driven process rather than usual interstitial pneumonia. Honeycombing, fibroblast foci, and smooth muscle hyperplasia can occur in idiopathic pulmonary fibrosis and are less specific.

Question 38Diffuse Parenchymal/Interstitial Lung Disease

A 68-year-old heavy smoker has upper-lobe emphysema and lower-lobe fibrosis. Lung volumes are relatively preserved, but the diffusing capacity is disproportionately reduced. Which syndrome best fits these findings?

  1. Chronic obstructive pulmonary disease alone
  2. Pure idiopathic pulmonary fibrosis
  3. Asbestosis with pleural plaques
  4. Combined fibrosis and emphysema Correct answer.

Explanation

Combined pulmonary fibrosis and emphysema is characterized by upper-lobe emphysema and lower-lobe fibrosis, with preserved or only mildly reduced lung volumes but a markedly reduced diffusing capacity. The combination reflects opposing effects on lung mechanics. Pure idiopathic pulmonary fibrosis, COPD alone, and asbestosis do not explain the full pattern.

Question 39Diffuse Parenchymal/Interstitial Lung Disease

A 40-year-old woman has recurrent pneumothorax, multiple lower-lobe and mediastinal lung cysts, and a family history of renal tumors. Which gene is most likely involved?

  1. FLCN Correct answer.
  2. SFTPC
  3. MUC5B
  4. TSC2

Explanation

Birt-Hogg-Dubé syndrome is caused by FLCN mutations and is characterized by lower-lobe and mediastinal cysts, recurrent pneumothorax, and renal tumors. The family history and cyst distribution are typical. TSC2, SFTPC, and MUC5B are associated with different cystic or interstitial lung diseases.

Question 40Diffuse Parenchymal/Interstitial Lung Disease

Which genetic variant is most strongly associated with increased risk of both familial and sporadic idiopathic pulmonary fibrosis?

  1. MUC5B promoter polymorphism Correct answer.
  2. Alpha-1 antitrypsin deficiency allele
  3. CFTR mutation with bronchiectasis
  4. HOXB cluster duplication

Explanation

A common MUC5B promoter polymorphism is the strongest genetic risk factor for familial and sporadic idiopathic pulmonary fibrosis. It is associated with increased MUC5B expression in distal airways. CFTR, alpha-1 antitrypsin, and HOXB variants are not the principal risk variants for idiopathic pulmonary fibrosis.

Question 41Pulmonary Vascular Disease

Which mean pulmonary arterial pressure at rest, measured by right heart catheterization, defines pulmonary hypertension under the current hemodynamic definition?

  1. ≥35 mm Hg
  2. ≥30 mm Hg
  3. ≥25 mm Hg
  4. ≥20 mm Hg Correct answer.

Explanation

Pulmonary hypertension is currently defined hemodynamically as a mean pulmonary arterial pressure of at least 20 mm Hg at rest. The older threshold of 25 mm Hg is no longer the defining value. Higher thresholds are not used to define pulmonary hypertension.

Question 42Pulmonary Vascular Disease

Idiopathic pulmonary arterial hypertension belongs to which WHO clinical group of pulmonary hypertension?

  1. Group 1 Correct answer.
  2. Group 4
  3. Group 2
  4. Group 3

Explanation

WHO group 1 comprises pulmonary arterial hypertension, including idiopathic, heritable, drug- and toxin-associated, and associated forms. Group 2 is pulmonary hypertension due to left heart disease. Group 3 is due to lung disease or hypoxemia, and group 4 is chronic thromboembolic pulmonary hypertension.

Question 43Pulmonary Vascular Disease

A 45-year-old woman presents with pleuritic chest pain and dyspnea. She has no history of venous thromboembolism, heart rate is 88/min, and she has no hemoptysis, estrogen use, surgery in the past 4 weeks, or signs of deep vein thrombosis. Pretest probability for pulmonary embolism is low. Which is the most appropriate next step?

  1. Proceed directly to CT pulmonary angiography
  2. Obtain a ventilation-perfusion scan
  3. Obtain a D-dimer Correct answer.
  4. Start therapeutic anticoagulation empirically

Explanation

In a patient with low pretest probability and no contraindication, a D-dimer is the appropriate initial test; a negative result can exclude pulmonary embolism without imaging. CT pulmonary angiography is reserved for patients with higher pretest probability or a positive D-dimer. Empirical anticoagulation before diagnosis is not appropriate in a stable patient.

Question 44Pulmonary Vascular Disease

A 60-year-old man with acute pulmonary embolism has blood pressure 78/50 mm Hg, heart rate 130/min, and lactate 4.2 mmol/L. Which treatment is most appropriate?

  1. Oral rivaroxaban alone
  2. Observation and repeat echocardiography in 24 hours
  3. Inferior vena cava filter placement
  4. Systemic thrombolysis Correct answer.

Explanation

Hemodynamic instability defines high-risk pulmonary embolism, and reperfusion therapy is indicated. Systemic thrombolysis is first-line when there is no contraindication. Anticoagulation alone is insufficient in this setting, and an inferior vena cava filter is not a primary reperfusion strategy.

Question 45Pulmonary Vascular Disease

Which medication is a phosphodiesterase-5 inhibitor used to treat pulmonary arterial hypertension?

  1. Epoprostenol
  2. Bosentan
  3. Ambrisentan
  4. Sildenafil Correct answer.

Explanation

Sildenafil and tadalafil are phosphodiesterase-5 inhibitors that increase cyclic guanosine monophosphate and promote pulmonary vasodilation. Bosentan and ambrisentan are endothelin receptor antagonists. Epoprostenol is a prostacyclin analogue.

Question 46Pulmonary Vascular Disease

Chronic thromboembolic pulmonary hypertension is diagnosed when pulmonary hypertension persists despite how long of anticoagulation after acute pulmonary embolism?

  1. 3 months Correct answer.
  2. 12 months
  3. 1 month
  4. 6 months

Explanation

Chronic thromboembolic pulmonary hypertension is considered when pulmonary hypertension and persistent perfusion defects remain after at least 3 months of therapeutic anticoagulation. Shorter intervals are insufficient to distinguish acute residual thrombus from chronic disease. Waiting 6 or 12 months is not required before evaluation.

Question 47Pulmonary Vascular Disease

In a patient with idiopathic pulmonary arterial hypertension undergoing right heart catheterization, which result defines a positive acute vasoreactivity test?

  1. A rise in mean pulmonary arterial pressure of ≥10 mm Hg with a fall in cardiac output
  2. A fall in pulmonary vascular resistance of ≥20% with a fall in mean pulmonary arterial pressure of 5 mm Hg
  3. A fall in mean pulmonary arterial pressure of ≥10 mm Hg to an absolute mean pulmonary arterial pressure ≤40 mm Hg with preserved or increased cardiac output Correct answer.
  4. A fall in mean pulmonary arterial pressure of ≥10 mm Hg without any change in cardiac output

Explanation

A positive acute vasoreactivity test requires a fall in mean pulmonary arterial pressure of at least 10 mm Hg to an absolute value of 40 mm Hg or less, with unchanged or increased cardiac output. This identifies the small subset of patients who may respond to calcium channel blockers. A fall in pressure alone or a rise in pressure does not qualify.

Question 48Pulmonary Vascular Disease

A 32-year-old woman with idiopathic pulmonary arterial hypertension has a positive acute vasoreactivity test. She has no evidence of right heart failure and is not pregnant. What is the most appropriate initial therapy?

  1. High-dose calcium channel blocker Correct answer.
  2. Observation without therapy
  3. Pulmonary endarterectomy
  4. Continuous intravenous epoprostenol

Explanation

Patients with idiopathic pulmonary arterial hypertension and a positive vasoreactivity test may be treated initially with high-dose calcium channel blockers and close reassessment. If they do not improve or maintain response, additional pulmonary arterial hypertension therapy is indicated. Pulmonary endarterectomy is for chronic thromboembolic disease, not idiopathic pulmonary arterial hypertension.

Question 49Pulmonary Vascular Disease

Which patient should undergo annual screening with transthoracic echocardiography for pulmonary hypertension?

  1. A 60-year-old with chronic obstructive pulmonary disease and mild dyspnea
  2. A 30-year-old with asthma and normal spirometry
  3. A 40-year-old man with treated obstructive sleep apnea and a normal echocardiogram
  4. A 55-year-old woman with limited cutaneous systemic sclerosis Correct answer.

Explanation

Systemic sclerosis, particularly the limited cutaneous form, carries a substantial risk of pulmonary arterial hypertension, and annual transthoracic echocardiography is recommended for screening. Obstructive sleep apnea, asthma, and chronic obstructive pulmonary disease may cause group 3 pulmonary hypertension but do not warrant routine annual echocardiographic screening in the same way.

Question 50Pulmonary Vascular Disease

A patient with severe chronic obstructive pulmonary disease and echocardiographic signs of pulmonary hypertension has mean pulmonary arterial pressure 32 mm Hg and pulmonary vascular resistance 3.5 Wood units. What is the preferred initial management?

  1. Refer for pulmonary endarterectomy
  2. Start ambrisentan
  3. Optimize oxygen and treat the underlying lung disease Correct answer.
  4. Start riociguat

Explanation

Pulmonary hypertension due to lung disease belongs to WHO group 3, and management centers on treating the underlying lung disease and correcting hypoxemia. Pulmonary arterial hypertension-specific vasodilators are not routinely recommended and may worsen ventilation-perfusion mismatch. Pulmonary endarterectomy is reserved for chronic thromboembolic pulmonary hypertension.