60 ABIM Pulmonary Disease Board Exam Practice Questions

Referral to a thoracic surgeon for consideration of video-assisted thoracoscopic surgery with lung biopsy is correct. This patient has rheumatoid arthritis (RA) and evidence of interstitial lung disease (ILD). RA is associated with several different forms of ILD, including usual interstitial pneumonitis (UIP), nonspecific interstitial pneumonitis (NSIP), and bronchiolitis-obliterans organizing pneumonitis (BOOP). The presence of CT changes suggestive of UIP portends a very bad prognosis, and consideration of lung transplantation in a specialized center which performs transplants for higher-risk patients with connective tissue disease may be the only curative therapy. This patient’s scan shows interstitial lung disease with evidence of fibrosis (without honeycombing and upper-lobe predominance) on the CT scan. The lack of honeycombing and basilar predominance makes the scan atypical for UIP. When a scan is typical of UIP, there is approximately 95% concordance with surgical lung biopsy specimens, making a biopsy unnecessary in many of these cases. Given the lack of typical findings on this patient’s CT scan, in addition to the nondiagnostic findings on transbronchial lung biopsy, a surgical lung biopsy is indicated; thus, referral to a thoracic surgeon for further evaluation is correct.

Watchful waiting with reevaluation in the clinic and pulmonary function testing in 6 weeks is incorrect. The patient has progressive symptoms and findings on chest imaging. Watchful waiting is likely to delay diagnosis and lead to worsening lung function prior to making this diagnosis.

Repeat bronchoscopy with use of electromagnetic navigation to reach the most affected areas on CT scan is incorrect. The limitation of transbronchial lung biopsy in definitively diagnosing interstitial lung disease (ILD) is the small size of a tissue sample without the acquisition of sufficient material to discern the underlying pattern of disease necessary for diagnosis.

Initiation of prednisone 1 mg/kg daily for 4 weeks followed by reevaluation in clinic is incorrect. Empiric immunosuppression may expose the patient to unnecessary morbidity of therapy and may delay appropriate diagnosis as response times can be long and are highly variable-even when the condition does respond favorably.

Referral back to rheumatologist for consideration of change in her rheumatoid arthritis therapy is incorrect. It is not clear that the interstitial lung disease (ILD) will respond to immunosuppression. Usual interstitial pneumonitis (UIP) will progress despite effective disease control. Establishing a diagnosis is critical in knowing how to best manage the patient’s immunosuppression.

Initiate nebulized DNAse once daily is correct as inhaled DNAse has been shown to improve lung function and decrease rates of exacerbations in patients with cystic fibrosis. This effect is achieved by facilitating airway clearance.

Initiate nebulized budesonide 500 mcg twice daily is incorrect. The addition of inhaled steroids does not achieve significant improvement in cystic fibrosis patients unless there is an indication of concomitant asthma.

Initiate inhaled fluticasone 250 mcg:salmeterol 50 mcg twice daily is incorrect as inhaled fluticasone-salmeterol is also a therapy for asthma and does not benefit patients with cystic fibrosis who do not have concomitant asthma.

Initiate inhaled tobramycin twice daily is incorrect because inhaled antibiotics are of specific benefit in patients with chronic pseudomonas colonization. This patient’s microbiologic history is not known from the information above.

Initiate inhaled aztreonam 3 times a day is incorrect because inhaled antibiotics are of specific benefit in patients with chronic pseudomonas colonization. This patient’s microbiologic history is not known from the information above.

Normal PaO2, low PaCO2 is correct because pink puffers have increased alveolar ventilation. They are breathless but not cyanosed and usually progress to type one respiratory failure. The respiratory centers remain sensitive to CO2, and therefore supplemental oxygen can be given. Blue bloaters, on the other hand, have a decreased alveolar ventilation. They rarely become breathless, but cyanosis and right heart failure are common.

Decreased alveolar ventilation is incorrect because alveolar ventilation is increased in pink puffers.

Low PaO2, high PaCO2 is incorrect because it is a feature of blue bloaters. The respiratory centers are insensitive to CO2, and hypoxia is the main drive to carry out the respiratory effort. Therefore, supplemental oxygen should be given with caution in blue bloaters.

Cyanosis is incorrect because pink puffers are breathless but not cyanosed.

Absence of breathlessness is incorrect because dyspnea occurs in pink puffers due to increased alveolar ventilation.

Volume cycled is the correct option and represents the correct ventilator settings displayed by an expiratory flow that occurs only when a preset volume is achieved.

Pressure cycled is an incorrect option as this represents expiratory flow occurring when a preset net pressure is achieved. This would be displayed as expiratory flow occurring at the same net pressure in succeeding cycles.

Time cycled is an incorrect option as this represents expiratory flow occurring at a regular interval determined by the settings on the ventilator.

Volume triggered is an incorrect option as this represents inspiration occurring when a preset volume is achieved. As the settings described in the question stem are that of expiratory flow, this cannot represent volume triggered ventilation.

Pressure triggered is an incorrect option as this represents inspiration occurring when a preset pressure is achieved. As the settings described in the question stem are that of expiratory flow, this display cannot represent pressure triggered ventilation.

Intermittent mandatory ventilation is the correct option as this is a mode where the machine continues to deliver positive-pressure breaths at a preset rate and any inspiratory effort by the patient between these breaths results in a spontaneous breath that is not delivered by the ventilator.

Controlled mechanical ventilation is an incorrect option as this mode is characterized by ventilation delivered at a preset rate. In this mode, the patient is unable to take spontaneous breaths.

Assist-control ventilation is an incorrect option as this mode is characterized by ventilation that occurs when either an inspiratory effort by the patient is detected or if a preset time has passed where the patient did not show any inspiratory effort.

Pressure-support ventilation is an incorrect option as this mode is characterized by a preset pressure supplemented by spontaneous inspiratory efforts made by the patient. In this way, the respiratory rate and tidal volume are determined by a combination of the pressure settings on the ventilator and the patient’s inspiratory efforts.

Negative-pressure ventilation is an incorrect option as this is a type of ventilation whereby negative pressure is utilized for the expansion and contraction of the chest cavity to simulate respiration.

Normal residual volume (RV) is correct because lung volumes and RV are preserved in chronic bronchitis, unlike emphysema, in which air trapping causes an increase in RV. The pulmonary function test (PFT) in chronic bronchitis suggests decreased FEV1/FVC, normal lung volume, RV, static lung compliance, diffusion capacity, increased PaCO2, and decreased gas exchange. In contrast, PFT in emphysema shows the obstructive pattern of air trapping with preserved gas exchange and PaCO2. Diffusion capacity is decreased, and static lung compliance is increased in patients with emphysema.

Signs of right heart failure is incorrect because cor pulmonale can be seen with both chronic bronchitis and emphysema.

Smoking history is incorrect because chronic heavy smoking is the major risk factor for chronic bronchitis and emphysema.

Family history of obstructive lung disease is incorrect because the family history of chronic obstructive pulmonary disease (COPD) is not characteristic of either emphysema or chronic bronchitis.

Decreased FEV1/FVC is incorrect because both chronic bronchitis and emphysema are characterized by decreased FEV1/FVC on pulmonary function test (PFT).

pH 7.50, PaO2 96 mm Hg, PaCO2 41 mm Hg, HCO3- 32 mEq/L is correct. The present case describes a patient with Bartter syndrome and therefore arterial blood gas analysis is most likely to show pH above 7.45, normal PaO2, normal PaCO2, and high HCO3- level. Bartter syndrome is an autosomal recessive disorder characterized by hypokalemia and hypochloremic metabolic alkalosis. Clinical features such as polydipsia, polyuria, vomiting, constipation, and failure to thrive and laboratory findings suggest the diagnosis. High urinary excretion of calcium and normal urinary excretion of magnesium are characteristic of all types of Bartter syndrome, except type V. Flattened T waves and prominent U waves in electrocardiogram are due to hypokalemia. Bartter syndrome is an important cause of chloride unresponsive metabolic alkalosis.

pH 7.31, PaO2 85 mm Hg, PaCO2 49 mm Hg, HCO3- 24 mEq/L is incorrect because these findings suggest respiratory acidosis and the patient has no condition predisposing to respiratory acidosis.

pH 7.32, PaO2 92 mm Hg, PaCO2 40 mm Hg, HCO3- 18 mEq/L is incorrect because the findings suggest metabolic acidosis.

pH 7.38, PaO2 97 mm Hg, PaCO2 38 mm Hg, HCO3- 25 mEq/L is incorrect because the findings are normal and do not indicate an acid-base abnormality.

pH 7.48, PaO2 90 mm Hg, PaCO2 27 mm Hg, HCO3- 23 mEq/L is incorrect because the findings suggest respiratory alkalosis.

Chronic obstructive pulmonary disease (COPD) is correct because the patient in this scenario has an onset of symptoms at a late age and has a smoking history. The pulmonary function test (PFT) shows obstructive lung disease with no significant post-bronchodilator response, which is a hallmark of COPD. COPD is characterized by exertional dyspnea and cough that occurs late in life in people who have a history of tobacco smoking or exposure to environmental pollutants. PFT shows mainly irreversible airflow limitation.

Asthma is incorrect because the history, symptoms, and pulmonary function test (PFT) result in this patient favor chronic obstructive pulmonary disease (COPD). A history of atopy in this patient may point towards asthma, but other features of this patient’s disease negate this diagnosis. Asthma is the major differential for COPD and distinguishing asthma from COPD remains a clinical challenge. Early age of onset of disease, history of atopy, family history of asthma, nighttime symptoms, and significant bronchodilator response on PFT are the features of asthma.

Asthma-COPD overlap syndrome (ACOS) is incorrect because ACOS is considered in patients whose symptoms fluctuate and do not diagnose either asthma or chronic obstructive pulmonary disease (COPD). ACOS is diagnosed by a 15% increase in FEV1 as a post-bronchodilator response.

Alpha-1 antitrypsin (AAT) deficiency is incorrect because it is characterized by features of chronic obstructive pulmonary disease (COPD) and liver cirrhosis occurring in a middle age without evidence of tobacco smoking.

Eosinophilic granulomatosis with polyangiitis (EGPA) is incorrect because it is characterized by a history of asthma, fever, malaise, weight loss, and elevated eosinophil.

The relative risk of falling asleep in sepsis lecture is 20% is correct. Relative risk is the ratio of the probability of an event occurring in an exposed group to the probability of the event occurring in a comparison, non-exposed group. The relative risk of falling asleep in a sepsis lecture is calculated as follows: (10/1000)/ (50/1000) = 0.2 (or 20% by taking this in percentage form i.e. 0.2Ã 100/100). Hence the relative risk of falling asleep in the sepsis lecture is 20% is correct.

The incidence of falling asleep in the sepsis lecture is 80% is incorrect. Incidence is a measure of the probability of occurrence of a given event or a medical condition in a population within a specified period of time. It can be expressed simply as the number of new cases during some time period but, it is better expressed as a proportion or a rate with a denominator. In this question, the incidence of falling asleep in the sepsis lecture will be 10/1000=1%. Therefore the incidence of falling asleep in the sepsis lecture is 80% is incorrect.

The relative risk of falling asleep in the sepsis lecture is 4% is incorrect.

No one falls asleep during statistics lectures is incorrect because 50 people fell asleep during the given statistics lecture.

Initiate tiotropium 18 mcg daily is correct. This patient has both underlying cardiac and respiratory disease. Cardiopulmonary exercise testing can help elucidate a patient’s symptoms by determining the degree to which limitation is related to cardiac or ventilatory processes. This patient’s test reveals evidence of a ventilatory limitation. Ventilatory reserve (1-VEmax/MVV) is reduced at 10% (normal >25%-30%). The lack of an anaerobic threshold and reduced peak heart rate are also suggestive of exercise which is halted due not to a cardiac limitation but rather respiratory cause before the circulatory system can be stressed to the point of anaerobic metabolism and elevated peak heart rates. The patient is taking a beta-blocker; however, while this may reduce peak heart rate, it does not impact the measurement of the anaerobic threshold. The etiology of the respiratory cause of dyspnea in a patient with chronic obstructive pulmonary disease (COPD) reflects obstruction to flow and gas trapping with hyperinflation. Tiotropium will help to reduce obstruction and thus, may prevent or delay gas trapping.

Increase concentration of inhaled fluticasone is incorrect as this will not directly decrease obstruction. Decreasing inflammation and thus, possibly airway caliber is an indirect process, and there is no evidence that the patient has significant inflammation from the information presented.

Obtain high-resolution CT scan of the chest is incorrect. The patient has known chronic obstructive pulmonary disease (COPD) and a high-resolution chest CT scan for the diagnostic purposes of evaluating a ventilatory limitation to exercise is unnecessary.

Initiate lisinopril 10 mg daily is incorrect. The patient does not have a cardiac limitation to exercise on testing and thus, interventions to help improve cardiac output through inotropic effect (digoxin) or peripheral vasodilatation (lisinopril), respectively, are not expected to benefit.

Suction the endotracheal tube is correct because with an elevated airway resistance, it is most likely that the endotracheal tube or proximal airways are plugged. The monophonic wheeze on exam is evidence in support of this. Suctioning may immediately relieve the acute respiratory distress.

Place bilateral chest tubes is incorrect because bilateral chest tubes would be indicated for bilateral pneumothorax; however, this would be associated with decreased respiratory system compliance-elevated plateau pressures-which were not observed with inspiratory hold.

Order stat chest X-ray is incorrect because this will delay treatment, and an endotracheal tube plug may not be seen on chest X-ray.

Perform bedside bilateral thoracic ultrasound is incorrect because bedside ultrasound may be helpful at detecting either pneumothorax or pleural effusion, neither of which are suspected as reviewed above.

Administer nebulized albuterol is incorrect because although nebulized albuterol will help relieve acute bronchospasm, the monophonic wheeze suggests a proximal obstruction which would not be expected to respond to bronchodilator therapy.

IgG-mediated immune complex depositions is correct. The patient given in the scenario is presenting with signs and clinical features of hypersensitivity pneumonitis. It is a granulomatous interstitial pulmonary disease that is common in patients with a history of prolonged exposure to organic irritants such as moldy hay. This exposure results in a mixed type III/type IV hypersensitivity reaction that causes IgG-mediated immune complex deposition in the lungs.

Elastase-mediated parenchymal destruction is incorrect as that is seen in emphysema due to alpha-1-antitrypsin deficiency. Patients present with clinical features of obstructive lung disease.

Aspergillus-induced eosinophil release is incorrect as that is seen in patients of allergic bronchopulmonary aspergillosis (ABPA), which clinically resembles asthma and again presents with clinical features of obstructive lung disease. Also, an x-ray of such patients will show dilated bronchi and/or branching opacities (finger in glove sign).

Silica-induced macrophage activation is incorrect as that is only seen patients with a history of exposure to the hazard such as coal workers.

IgE-mediated histamine release is incorrect as that is a defining trait of atopic conditions such as allergic rhinitis, asthma, and atopic dermatitis. Asthma will present with features of obstructive lung disease.

Ethnicity is the correst option. The patient in the question stem is presenting with features that are highly suggestive of sarcoidosis, i.e., noncaseating granuloma, bilateral hilar lymphadenopathy, elevated CD4:CD8 ratio. This disease has 2 key epidemiological groups in which it is prevalent: African-Americans and women between age groups 25-35 and 45-65. Therefore, an African-American woman presenting with such symptoms of this age should raise suspicion for sarcoidosis.

Exposure to acid-fast bacilli is incorrect as that predisposes the patient to pulmonary tuberculosis, which will result in the patient having caseating granulomas but as given in the question stem, this is not the case.

Exposure to beryllium is incorrect as that predisposes to berylliosis. In such patients, BAL would show lower CD4:CD8 ratio along with mast cells, foam cells, and plasma cells.

Alcohol consumption is associated with aspiration pneumonia rather than sarcoidosis, and that has a different presentation than the one of this patient; therefore, it is incorrect.

Smoking is incorrect as that is a risk factor for the development of cancer. The patient does have some features of malignancy, but cancer does not explain her lupus pernio or BAL findings. Also, smoking does not predispose to the development of sarcoidosis.

Bronchiolitis obliterans is correct because the spirometry in bronchiolitis obliterans demonstrates an obstructive ventilatory defect with a progressive decline in the forced expiratory volume over 1 second (FEV1) and a decrease in the ratio of FEV1 to forced vital capacity (FVC), which is decreased in this patient to 37%. Bronchiolitis obliterans is a clinical syndrome marked by progressive dyspnea and cough with the absence of parenchymal lung disease on radiographic studies. Pulmonary function testing reveals an obstructive ventilatory defect that is typically not reversed by inhaled bronchodilator.

Interstitial fibrosis is incorrect because spirometry in interstitial fibrosis shows that the FEV1 and FVC are proportionally reduced, thus preserving the FEV1/FVC ratio, unlike this patient.

New-onset asthma is incorrect because, even though there is an obstructive defect, asthma would be less likely to present for the first time at this age. Bronchodilator testing would also reveal improvement. (1-4)

Methotrexate-induced lung disease is incorrect because methotrexate was not associated with an increased risk of total adverse respiratory events.(1-5)

Pulmonary vasculitis is incorrect because the FEV1/FVC is not lowered in such disease.

Intranasal interferon spray is correct. According to different researches, many targets have been identified to formulate a vaccine or certain drugs which can be effective in preventing rhinovirus infection, but due to ever-increasing serologic variants and uncertainty about the immunologic processes, the efficacy and potential of these vaccines are questionable.

Vaccination is incorrect. A clinical trial indicated the effective role of intranasal human interferon alpha-2 in preventing common colds caused by rhinovirus in sub-populations and reducing the severity of symptoms, but a major confounder was the irritation of nasal mucosa.

Pleconaril is an antivirus drug which inhibits the viral attachment to the host by binding to the viral capsid. It has yielded mixed results so not commonly used for cases of rhinovirus prevention; instead, they might be more useful in the prevention of enterovirus.

Prophylactic antibiotics have no role in preventing diseases caused by viruses. Antibiotics may be used when a superinfection with bacteria, for example, otitis media over rhinovirus infection is suspected.

Nasal decongestants are used in the treatment of nasal congestion caused by rhinovirus as a symptom, but they have no preventive role.

Inhaled Dornase (DNAse) therapy is not recommended for patients with non-CF bronchiectasis and having primary ciliary disease, for the reason that it induces more frequent pulmonary exacerbations and the forced expiratory volume in one second (FEV1) greatly declines, so this option is correct.

Airway clearance with inhaled mannitol (dry powder form) greatly reduces exacerbations, and, therefore, this answer is also incorrect.

Azithromycin three times weekly is incorrect. It is a macrolide antibiotic that has both antimicrobial and immunomodulatory properties and controls pulmonary exacerbations when taken in doses of 500mg three days per week, so this answer is incorrect as well.

Chest physiotherapy is one mechanical intervention used in symptomatic bronchiectasis for improving the mucous airway clearance for preventing mucous plugging and preventing airway inflammation and recurrent infections, so this answer is incorrect.

Pulmonary arterial hypertension is correct. It accounts for a heterogeneous group of disorders with the common feature of increased pulmonary vascular resistance and narrowing of blood vessels within the pulmonary vasculature. Patients usually present with vague symptoms of worsening weakness and dyspnea on exertion.

Pulmonary embolism is an incorrect option as there is a normal perfusion lung scan.

Interstitial pneumonia is incorrect as it is a restrictive lung disease. It is also incorrect because of the normal chest x-ray.

Sarcoidosis is incorrect as it is a restrictive lung disease. It is also incorrect because of the normal chest x-ray.

Left ventricular diastolic dysfunction is incorrect as even though pulmonary hypertension may occur due to left heart disease, there is no indication that it is occurring here. With left heart disease, one would more likely see pulmonary edema which was not seen on x-ray.

ALOX5AP is correct as the patient seems to be having aspirin induced asthma evidenced by dyspnea and chest tightness which developed immediately after taking the medication. While aspirin is the main medicine involved, non-steroidal anti-inflammatory drugs such as ketorolac can also induce it. ALOX5AP gene transcription has been shown to be increased in aspirin induced asthma.

CTRL-1 is incorrect as there has not been any studies linking it to the development of aspirin induced asthma.

HLA-DQW4 is incorrect as no link has been seen yet between this gene and aspirin induced asthma. HLA-DQW2 is however, related to this condition.

HLA-DPB3 is incorrect as there is no known link between this gene and aspirin induced asthma. However, HLA-DPB1*0301 and HLA-DPB1*0401 have been shown to have some association with asthma.

TBX12 is incorrect as no association between this gene and aspirin induced asthma has been observed.

Enoxaparin is correct as the patient has had cancer surgery. Enoxaparin 40 mg daily for approximately a month is recommended as a prevention strategy for venous thromboembolism in hospitalized patients who have had a cancer surgery including gynecologic cancer surgery.

Dabigatran is incorrect as the patient has just had a recent surgery for a gynecologic cancer. Dabigatran is preferred in patients who have had a major orthopedic surgery.

Intermittent pneumatic compression device is incorrect as the patient does not have any contraindication to anticoagulation and has not had a major orthopedic surgery. Given her history of malignant disease, this patient requires pharmacologic therapy to prevent development of thrombi. Contraindication to anticoagulation and a major orthopedic surgery are conditions in which intermittent pneumatic compression device is used.

Unfractionated heparin is incorrect as the patient has had a recent gynecologic cancer surgery. Unfractionated heparin is usually used in hospitalized patients who have had a high risk non-orthopedic surgery. In patients with cancer surgery, enoxaparin is preferred.

Warfarin is incorrect as the patient has not had a major orthopedic surgery, in which warfarin is used.

Ciprofloxacin + metronidazole is correct. The patient presenting in the given scenario has disease that predisposes to aspiration pneumonia and requires management with appropriate antibiotic therapy with activity against gram-negative organisms. Third-generation cephalosporins fluoroquinolones or piperacillin-tazobactam will be effective against such organisms.
Ciprofloxacin + gentamicin is incorrect. Gentamicin is also appropriate for combating enteric gram-negative bacteria, but it is not the recommended choice especially as there are potential toxicities and metronidazole would be preferred; therefore, the option ciprofloxacin + gentamicin is not the best choice.
Piperacillin/tazobactam will not be given to the patient due to her penicillin allergy; therefore, this option is incorrect.
Azithromycin has good coverage of atypical causes of community-acquired pneumonia, but spectrum is otherwise limited; therefore, this option is incorrect.
Imipenem will not be given to the patient due to her allergy; therefore, this option is incorrect.

No prophylaxis needed is correct. According to the risk stratification by the American College of Physicians, a patient aged between 41 and 60 years is a risk score of 1, and being the only risk in this patient, a total risk score of 1 (low-risk category) requires no prophylaxis for DVT.

Enoxaparin 40mg SQ daily is incorrect because it is not indicated in this patient as he has no risk factors for thromboembolism.

Heparin 5000 units SQ BID is incorrect because it is not indicated in a patient without any risk of thromboembolism.

Fondaparinux 2.5mg SQ BID is incorrect because it is not indicated in this patient who has no risk of thromboembolism.

Heparin 5000 units SQ TID is incorrect because it is not indicated in this patient without a risk factor for thromboembolism and may increase the risk of bleeding in this patient.

Primary pulmonary hypertension is correct as this patient stopped exercise (after 8 minutes) because of dyspnea, and this is considered a classical sign of primary pulmonary hypertension. Nearly all pulmonary function abnormalities are present in this female patient such as reduction in AT, VO2 max, O2 pulse, SaO2, and peak O2. The maximum heart rate (HR) is normal in primary pulmonary hypertension.

Interstitial lung disease from sarcoidosis is incorrect as patients with interstitial lung disease from sarcoidosis may have disproportionate cardiovascular abnormalities. No ECG changes were seen in this female patient. The AT response is normal or slightly reduced in interstitial lung disease. In this patient, AT is markedly reduced. HR max is normal in this female patient, whereas HR max is reduced in interstitial lung disease. The O2 pulse is normal or slightly reduced in interstitial lung disease, whereas it is significantly reduced in this patient.

Deconditioning is incorrect as the AT is normal or slightly reduced in deconditioning, whereas the AT is markedly reduced in this patient. The SaO2 is normal in deconditioning, whereas the SaO2 is markedly low in this patient.

Exercise induced asthma is incorrect as, in exercise-induced asthma patients, the O2 saturation should be normal at rest, and below normal with exercise. In this female patient, the O2 saturation is below the normal range at rest.

Poor effort is incorrect as normal or unattained AT is usually seen in poor effort, whereas in this patient, it is significantly low. The SaO2 is normal in poor effort, whereas the SaO2 is markedly low in this patient.

Epoprostenol is correct. Treatment of idiopathic pulmonary arterial hypertension (IPAH) includes the use of calcium channel blockers in patients with an acute vasodilatory response to intravenous prostacyclin analogs such as epoprostenol, and other IPAH-specific therapies such as Bosentan, an oral endothelin receptor antagonist, and Trepostinil, an intravenous or subcutaneous, parenteral prostacyclin analog. Therefore, a positive response to epoprostenol increases the survival odds for people with IPAH.

Calcium channel blockers is incorrect because they are only indicated in patients with acute vasodilatory response to intravenous epoprostenol, therefore they do not improve survival rates on their own.

Bosentan is incorrect as its use alone without a vasodilatory response to intravenous epoprostenol is associated with a poor prognosis.

Trepostinil is incorrect because its use alone without a vasodilatory response to intravenous epoprostenol is associated with a poor prognosis.

All of the above is incorrect because calcium channel blockers, Bosentan, and Trepostinil used independently without a positive response to epoprostenol are associated with low survival rates.

Administer high flow oxygen and reposition chest drain is the correct answer as this will lead to resorption of the air in the subcutaneous space in the majority of the cases.

Remove chest drain is incorrect as it will prevent the resolution of underlying pneumothorax, which can lead to an increase in the severity of this patient’s symptoms.

Administer analgesia and continue observation is incorrect as this will not cause resolution of the surgical emphysema.

Perform skin incision decompression is incorrect as this is indicated in patients with severe respiratory distress and upper airway obstruction due to severe surgical emphysema.

Refer to a thoracic surgeon is incorrect as it is indicated when the underlying pneumothorax does not resolve or there is a persistent air leak for more than 4 days.

Endotracheal aspiration with semiquantitative cultures is the recommended microbiologic method for the diagnosis of ventilator-associated pneumonia (VAP). Endotracheal aspiration is a non-invasive method of obtaining tracheal secretions for the culture and microbiologic diagnosis.

Bronchoscopy with quantitative cultures is incorrect.

Blind bronchial sampling with semiquantitative cultures is incorrect. Qualitative analyses indicate the presence or absence of growth of pathogens, semiquantitative analyses focus on grading the bacterial growth as categories (e.g., ± (scanty), + (few), ++ (moderate), and +++ (numerous)), and quantitative analyses indicate the exact bacterial growth count to differentiate between infection and colonization.

Endotracheal aspiration with qualitative cultures is incorrect. There is no evidence that in comparison to non-invasive sampling with either semiquantitative or quantitative cultures, invasive respiratory sampling with quantitative cultures leads to improved clinical outcomes. Moreover, semiquantitative cultures can be performed more rapidly compared to quantitative cultures, with lesser expertise and fewer laboratory resources required.

Bronchoscopy with qualitative cultures is incorrect. Non-invasive sampling (i.e., endotracheal aspiration) with semiquantitative cultures, rather than noninvasive sampling with quantitative or qualitative cultures, and rather than invasive sampling with quantitative or qualitative cultures, is generally recommended for the diagnosis of ventilator-associated pneumonia (VAP).

Needle thoracostomy, which decompresses the pleural space and relieves pressure on nearby structures, is the first-line treatment for tension pneumothorax.

Administer supplemental oxygen is appropriate for small, primary spontaneous pneumothoraces to treat hypoxemia and facilitate the absorption of trapped air from the pleural space. This intervention does not, however, acutely relieve the pressure that a tension pneumothorax places on nearby structures and will not resolve this patient’s cardiopulmonary distress.

Intravenous administration of fentanyl is incorrect. Although pain control is an important element of treating patients with gunshot wounds, the administration of pain control medications, such as fentanyl, should not delay more critical components of management.

Ultrasonography of the chest findings that show an absence of lung sliding (i.e., visceral pleura moving against the parietal pleura) suggest a pneumothorax. Imaging studies should not delay treatment of a tension pneumothorax.

Endotracheal intubation may be indicated in penetrating chest wall trauma to stabilize a patient’s airway and facilitate breathing. This intervention does not, however, relieve the cardiopulmonary distress caused by a tension pneumothorax.

pH <7.2 is correct as it indicates an exudative pleural effusion. This patient has a fever, pleuritic chest pain, cough, and dyspnea, suggesting an exudative effusion as the likely cause.

Increased amylase is incorrect as it is seen in patients with esophageal rupture, pancreatitis, or malignancy. This patient’s findings are consistent with a parapneumonic effusion which does not present with an increased pleural fluid amylase.

Glucose of 90 mg/dL is incorrect as pleural fluid glucose < 60 mg/dL suggests an exudate. This patient’s findings are consistent with parapneumonic effusion of an infectious etiology. Lymphocytes > 90% is incorrect as it is seen in patients with tuberculosis and certain malignancies like lymphoma. This patient’s signs and symptoms are not consistent with this finding.

Pleural fluid/serum LDH ratio of 0.5 is incorrect as this ratio is 0.6 for transudative effusions. This patient’s findings are consistent with an exudative parapneumonic effusion.

Increase positive end-expiratory pressure is correct as this patient is in severe hypoxemia (PaO2 <60 mm Hg) and bilateral opacities on chest x-ray suggestive of acute respiratory distress syndrome. Positive end-expiratory pressure (PEEP) improves oxygenation by recruiting more alveoli and increases ventilation by reducing shunting.

Keep current ventilator settings is incorrect as this patient has worsening hypoxemia despite current settings. Positive end-expiratory pressure (PEEP) should be increased to improve oxygenation and to allow for a reduction in FiO2.

Increase FiO2 is incorrect as it should be weaned to <60% quickly to prevent oxygen toxicity. This patient’s positive end-expiratory pressure (PEEP) should be increased to improve oxygenation.

Increase tidal volume is incorrect as this patient has proper ventilation. The increasing tidal volume will over distend the alveoli and increase the risk of barotrauma. This patient’s tidal volume is consistent with lung-protective ventilation.

Increase respiratory rate is incorrect as this patient’s ventilation is adequate. Increasing the respiratory rate will worsen the respiratory alkalosis without improving oxygenation in this case.

Increased pulmonary shunt fraction is correct. Accumulation of fluid in the alveolar space and a decrease in surfactant due to disruption by intra-alveolar neutrophils leads to alveolar collapse and impaired alveolar ventilation, while perfusion remains largely constant. The resultant V/Q mismatch leads to an increase in the amount of venous blood that bypasses oxygenation and reaches systemic circulation, thereby increasing the shunt fraction and causing this patient’s presentation of tachypnea and cyanosis.

Increased mixed venous oxygen saturation is incorrect. This patient’s acute respiratory distress syndrome (ARDS) causes V/Q mismatch, which leads to cyanosis and respiratory compromise. This patient’s cyanosis is due to decreased arterial oxygen saturation from hindered alveolar ventilation. Therefore, a decrease (not increase) in mixed venous oxygen saturation would be expected.

Increased pulmonary wedge pressure is the consequence of increased left atrial pressure from left-sided heart disease, and is associated with cardiogenic pulmonary edema. This patient, however, presents with acute respiratory distress syndrome (ARDS), which is defined by the presence of noncardiogenic pulmonary edema.

Increased pulmonary compliance is incorrect. This patient’s acute respiratory distress syndrome (ARDS) is the consequence of an inflammatory reaction that has led to severe pulmonary edema and disruption of surfactant, thus causing decreased (not increased) pulmonary compliance.

Decreased alveolar dead space is incorrect. Alveolar dead space is defined as the difference between physiologic dead space and anatomic dead space. In acute respiratory distress syndrome (ARDS), the fluid accumulation and decrease in surfactant cause alveoli to collapse. This collapse causes an increase in anatomic dead space (to a lesser degree) while it hinders or completely eliminates ventilation, thus increasing the amount of physiologic dead space (to a greater degree).

Thrombolysis with tissue plasminogen activator (tPA) is correct because this patient has developed high-risk pulmonary embolism (PE), as apparent from the right ventricular dysfunction, pulmonary hypertension, and systemic hypotension. There are no absolute contraindications present for thrombolysis. However, there are relative contraindications (current use of anticoagulation and remote history of cerebrovascular accident). Still, it is recommended to proceed with thrombolysis with tPA administered at 1mg/hr (limit total dose to less than 20 mg) because several studies demonstrate the superiority of systemic thrombolysis in improving right ventricular function. In addition, given persistent hypotension, this patient is at high risk, and the benefits of thrombolysis would outweigh the risks here.

Surgical embolectomy is incorrect because this patient has suffered from a high-risk pulmonary embolism (PE) with some relative contraindications to thrombolysis but no absolute contraindication. Surgical embolectomy is indicated in high-risk or intermediate-risk PE when there is saddle PE or embolus in bilateral main pulmonary arteries or intracardiac thrombus. This patient has none of these, so thrombolysis is a superior option to surgical embolectomy.

Inferior vena cava (IVC) filter is incorrect because the insertion of an IVC filter is indicated when there is a pulmonary embolism (PE) and the patient has absolute contraindications to the use of systemic thrombolysis. This patient has no absolute contraindications, so an IVC filter is an inferior treatment choice.

High-dose unfractionated heparin is incorrect because, in high-risk pulmonary embolism (PE), the first-line management strategy is systemic thrombolysis unless any absolute contraindication is present or there is a large bilateral physician assistant (PA) thrombus or intracardiac thrombus warranting embolectomy. High-dose unfractionated heparin with IVC filter may be considered in patients who have an absolute contraindication to thrombolysis and have developed high-risk PE.

Unfractionated heparin with high-dose warfarin is incorrect because, in high-risk pulmonary embolism (PE) with right ventricular dysfunction (RVD) and hypotension, unfractionated heparin combined with warfarin is inferior to systemic thrombolysis. Warfarin use has not been documented to be efficient in treating acute PE with RVD. Considering this patient has no absolute contraindications to thrombolysis present, it is recommended to proceed with intravenous tissue plasminogen activator (tPA) administration immediately.

Insertion of a chest tube is the correct option. Insertion of a chest tube into the 4th intercostal space at the midaxillary line is the most appropriate next step for treating pneumothorax in this patient. Mechanical ventilation (especially with a high positive end-expiratory pressure [PEEP]) is considered a risk factor for developing pneumothorax. High pressure can damage the lung tissue, which leads to a leak. This is known as Iatrogenic or traumatic pneumothorax which is a common complication in the ICU.

Needle decompression is incorrect because immediate insertion of a large-bore needle into the second intercostal space along the midclavicular line would be indicated for tension pneumothorax. In addition to the symptoms seen in this patient, tension pneumothorax also presents with cyanosis, contralateral tracheal deviation, distended neck veins, and significant hemodynamic instability (tachycardia, hypotension), none of which are present in this patient.

Administer levofloxacin is incorrect because respiratory fluoroquinolones such as levofloxacin are the first-line treatment in patients with hospital-acquired pneumonia who do not have risk factors for multi-resistant pathogens (prior antibiotic treatment, structural lung disease).

Increase the positive end-expiratory pressure (PEEP) is incorrect because it is often necessary when treating acute respiratory distress syndrome (ARDS). Patients with ARDS typically present with acute onset tachypnea, tachycardia, cyanosis, dyspnea, diffuse crackles, and possibly fever, cough, and chest pain. Chest x-ray of patients with ARDS usually reveals diffuse bilateral infiltrates, air bronchograms, atelectasis, and pleural effusions.

CT scan of the chest is incorrect because it is indicated in uncertain diagnosis despite chest x-ray, suspected underlying lung disease (to determine the likelihood of recurrent disease), and presurgical workup. The patient had a chest x-ray that showed deepening of the costophrenic angle, indicating a pneumothorax. Further workup with a chest CT is not needed at this time.

Low molecular weight heparin (LMWH), as a bolus, followed by a constant infusion for 4-5 days, is the initial treatment of choice because of its immediate antithrombotic effect and safety during breastfeeding. If no bleeding has occurred, it can safely be started 12 hours after the cesarean section.

Graduated compression stockings are indicated in the management of lymphedema since the compression of these tight-fitting stockings improve lymphatic drainage and decrease edema.

Fasciotomy is incorrect as this is the treatment of choice to relieve pressure, and thus, restore perfusion in patients with compartment syndrome.

Embolectomy is not indicated in the treatment of deep venous thrombosis (DVT) but may be considered for patients with acute limb ischemia (ALI) within the first 6-24 hours to restore arterial blood supply in a viable, nonthreatened limb.

Urokinase is a thrombolytic agent that is indicated in the treatment of pulmonary embolism, phlegmasia cerulea dolens, massive iliofemoral deep vein thrombosis (DVT), hemodynamic instability, and in patients who have failed anticoagulation.

Decreased shunting is correct. This patient requires mechanical ventilation secondary to pancreatitis-induced acute respiratory distress syndrome (ARDS). During mechanical ventilation, alveolar collapse or atelectasis can become a common problem. When large areas of the lungs are perfused but not ventilated, shunting occurs.

Increased minute ventilation allows patients to breathe off excess CO2 to reduce PCO2 quickly but has minimal effect on hypoxemia. In addition, increasing PEEP does little for minute ventilation.

Increased respiratory rate increases minute ventilation. It allows patients to breathe off excess CO2 to reduce PCO2 quickly but has minimal effect on hypoxemia.

Increased tidal volumes increase minute ventilation. Ventilation and oxygenation are two separate goals when it comes to mechanical ventilation. Increased ventilation can quickly breathe off excess CO2 to reduce PCO2 but has minimal effect on hypoxemia.

Increased FiO2 has a very limited effect on hypoxemia in conditions where hemoglobin is already maximally saturated, as seen in this patient.

Endotracheal intubation is correct. This patient is presenting with symptoms of a myasthenic crisis. He requires immediate intubation to prevent death due to respiratory failure.

Noninvasive ventilation with bilevel positive airway pressure is incorrect. It effectively manages myasthenia gravis patients presenting with signs of impending respiratory failure. However, it is effective in patients before the development of hypercapnia as it has been shown to shorten lengths of stay at the critical care unit. This patient has significant hypercapnia (>50 mmHg) and will not benefit from bilevel positive airway pressure.

Increase dose of pyridostigmine is incorrect and may result in a cholinergic crisis; this may further worsen his condition. Moreover, this patient needs to be intubated, and increasing doses of pyridostigmine will not alleviate his symptoms.

Chest physiotherapy is incorrect. There is no role for chest physiotherapy in managing a myasthenic crisis.

IV immunoglobulins is incorrect. Administration of IV immunoglobins is appropriate for managing an exacerbation of myasthenia gravis. However, this patient’s airway needs to be protected first.

Increase the positive end-expiratory pressure is the correct answer because this patient has an acute onset of hypoxemia with bilateral infiltrates on imaging. This presentation is concerning acute respiratory distress syndrome (ARDS), especially in the setting of his ongoing infection (wet gangrene). Increasing the PEEP will help distend the alveoli and decrease the pressure needed to inflate the lungs. Patients with severe ARDS often require higher levels of PEEP to help recruit alveoli and improve gas exchange. Since this patient is already at maximum FIO2 and is on appropriate lung-protective ventilation settings (tidal volume of 6 mL/kg), increasing PEEP would be the most appropriate next step in management.

Inhaled nitric oxide is incorrect because it is used in the treatment of certain patients with severe pulmonary hypertension by improving pulmonary vasodilation. This patient does not have any history to suggest a diagnosis of pulmonary hypertension (signs of right heart failure like edema) and it is not an effective treatment for acute respiratory distress syndrome (ARDS).

Prone positioning of the patient is incorrect; it may be indicated, but usually, it is only pursued after alternative interventions to stabilize the patient’s oxygenation have been attempted. If the oxygenation remains poor after appropriate ventilator adjustments then prone positioning may be considered.

Increase the tidal volume is incorrect because the high peak pressures and lung distention associated with high tidal volumes in patients with acute respiratory distress syndrome (ARDS) increase the risk of barotrauma and worsening respiratory distress. Using lower tidal volumes (lung-protective ventilation of around 6 mL/kg) improves mortality risk in patients with ARDS, and mild hypercapnia is tolerated in this regard (permissive hypercapnia).

Transfusion of red blood cells is incorrect because hypoxia in acute respiratory distress syndrome is a result of impaired gas exchange at the capillary-alveolar interface, so a blood transfusion will not improve his oxygenation. In addition, the hemoglobin is above a threshold where transfusion should be considered.

Tension pneumothorax is correct because of the typical findings seen on chest x-ray (mediastinal shifting towards the opposite side, intercostal spaces increased on the ipsilateral side) along with typical clinical findings (absent breath sounds on the ipsilateral side with hyper-resonant percussion sounds suggestive of air accumulation along with hemodynamic instability) in a patient with suspected viral pneumonia.

Traumatic pneumothorax is incorrect because the patient does not have any history of trauma.

Hemothorax is incorrect because in hemothorax, the affected side of the thorax usually gets opaque on chest x-ray and there is dullness on percussion, which is not seen in this case.

Chylothorax is incorrect because in chylothorax, the affected side of the thorax usually gets opaque on chest x-ray and there is dullness on percussion, which is not seen in this case.

Pleural effusion is incorrect because in pleural effusion, fluid is seen in the lung fissures on chest x-ray and there is dullness on percussion of the chest, which is not seen in this case.

Intravenous antibiotics for 7 days is the correct option. This patient has ventilator-associated pneumonia, which is defined as pneumonia that occurs more than 48 hours after intubation in the absence of any other causative factor. Early-onset is commonly due to antimicrobial-sensitive organisms instead of patients who develop pneumonia with multidrug-resistant organisms after more than a week of intubation. Current guidelines suggest a therapy of 7 days is adequate in uncomplicated pneumonia in the absence of rapid clinical deterioration or empyema. As the patient does not have fever, hypotension, and dyspnea, she will benefit from a shorter course of therapy.

Administer antibiotics for 2 weeks is incorrect because longer therapy leads to colonization with antibiotic-resistant bacteria, which may precede a recurrent episode of pneumonia.

Stop antibiotics when cultures become negative is incorrect because implicated organisms may colonize after complete treatment. Patients should be judged clinically and not by sputum culture.

Aerosolized antibiotics after a week is an incorrect option because these have not proven to benefit patients with ventilator-associated pneumonia. They have proven to be of some modest benefit as adjunctive therapy in patients with multidrug-resistant (MDR) gram-negative sepsis who poorly respond to systemic therapy.

Continue antibiotics until she is extubated is an incorrect option because current recommendations suggest antibiotic therapy for a week.

Joint pains and multiple first trimester abortions is the correct option. This patient has diffuse alveolar hemorrhage as evident by progressively bloody returns of bronchoalveolar lavage. There are numerous causes of diffuse alveolar hemorrhage, including vasculitis, renopulmonary syndromes, and antiphospholipid syndrome. It is important to understand the primary pathophysiology of the disorder so quick evaluation and prompt management can be undertaken. In the case of vasculitis, high-dose steroids and immunosuppressants like cyclophosphamide are effective. Plasmapheresis may also be tried in refractory cases.

Dry cough, bilateral hilar lymphadenopathy, and raised serum calcium is an incorrect option because these are present in sarcoidosis. It is a chronic granulomatous disease associated with erythema nodosum, hypercalcemia, and lymphocytosis on bronchoalveolar lavage.

Chronic hepatitis B is an incorrect option because chronic hepatitis C and associated cryoglobulinemia increase the risk of diffuse alveolar hemorrhage.

Polyarteritis nodosa is an incorrect option because it is a medium vessel vasculitis that seldom involves the lungs.

Aspirin for coronary artery disease is an incorrect option because antiplatelets are rarely associated with diffuse alveolar hemorrhage. It occurs when abciximab is given. Anticoagulants like warfarin may precipitate bleeding and hemorrhage.

Perform tube thoracostomy is the correct option. This patient has developed empyema as evident by a pleural fluid’s low pH, high protein, and low glucose. The recommended management of empyema includes broad-spectrum antibiotics and tube thoracostomy to provide drainage to the purulent pleural fluid. Any delay in management results in an increased risk of complications, mortality, and morbidity. A long duration of antibiotics is required in these patients.

Video-assisted thoracoscopic surgery is an incorrect option because it is needed when an effusion fails to resolve by tube thoracostomy or there is a multiloculated effusion.

Continue antibiotic therapy with serial imaging is incorrect because an empyema will not resolve without drainage

Discontinue cefepime and add meropenem is incorrect because broadening the antibiotic coverage will not resolve the infection without proper surgical drainage.

Repeat thoracentesis is incorrect because drainage with thoracentesis alone is inefficient because of the increased chance of fluid reaccumulating, making repeated drainage necessary.

Noninvasive ventilation is the correct option. This patient has acute on subacute shortness of breath. Her increased weight, type 2 respiratory failure, and nonurgent presentation are classic for obesity hypoventilation syndrome with right heart failure. As she has hypercarbia, she needs assisted ventilation. The patient should be given a trial of noninvasive ventilation, and if that fails, only then should she be intubated and mechanically ventilated. She needs echocardiography to assess pulmonary hypertension and right and left ventricular function. Moreover, she will benefit from weight loss.

Invasive mechanical ventilation is an incorrect option because invasive mechanical ventilation is associated with increased mortality and morbidity and therefore a trial of noninvasive ventilation is warranted first.

Nebulize with beta-agonists is an incorrect option because it treats asthma and chronic obstructive airway disease but would unlikely be helpful in this case.

IV hydrocortisone is incorrect because it has no role in the management of obesity hypoventilation syndrome.

IV furosemide is incorrect. Although her pedal edema suggests that she has developed cor pulmonale and may benefit from diuretics, it is more important to provide ventilatory support to the patient.

Switch to protective ventilation is the correct option. This patient has presented with respiratory failure due to almost complete airway obstruction. To overcome the obstruction, patients with upper airway obstruction may develop repeated negative airway pressure and negative pressure pulmonary edema. The result is the development of alveolar and interstitial edema, which disappears on initiation of positive pressure ventilation. In the presence of lung injury, the alveolar clearance rate may be reduced up to 25% of normal. A protective ventilation strategy can limit lung injury and improve the rate of alveolar clearance. The tidal volume should be 6 ml/kg, and plateau pressure should be less than 30 cm of water.

CT scan of the chest is an incorrect option because it should be performed after positive pressure ventilation, PEEP, and alveolar clearance reveal the actual damage caused to the lung tissue.

Fiberoptic bronchoscopy is an incorrect option because it will only show a light pink frothy transudate suggestive of pulmonary edema, which can easily be confirmed on tracheal aspiration.

Intravenous furosemide is an incorrect option because the efficacy of furosemide is hampered by lung injury.

Dexamethasone is an incorrect option because it treats high-altitude pulmonary edema and not negative pressure pulmonary edema.

Limiting end-expiratory pleural pressures to < -20 cm H2O is the correct option. This patient has developed re-expansion pulmonary edema as evident by tachycardia, hypoxia, and dyspnea occurring shortly after removing pleural effusion. It is unilateral pulmonary edema that occurs after re-expansion of the lung due to increased pulmonary vascular permeability, surfactant loss, and oxygen free radicals production. After drainage of pleural effusion or pneumothorax, there is increased blood flow and concurrent alveolar distention. The risk factors associated with this condition include prolonged obstruction or effusion and removal of more than 1.5 liters of fluid. Limiting end-expiratory pleural pressures to less than -20 cm H2O and gradual, staged drainage is the single most important protective factor. Treatment is supportive.

Administration of low molecular weight heparin before the procedure is incorrect because it is protective against thrombotic complications but does not prevent pulmonary edema.

IV albumin in a 1:1 ratio for fluid removed is incorrect because replacing albumin in patients with hypoalbuminemia may prevent rapid reaccumulation of transudative pleural effusion or ascites, but it would not be expected to help with pulmonary edema.

Increase the volume removed to 2.5 liters is incorrect because removal of more than 1.5 liters is associated with the development of unilateral pulmonary edema.

Systemic antibiotic before the procedure is incorrect because it is not recommended in thoracentesis.

Tracheostomy and physical therapy is the correct option. This patient has developed critical-illness myopathy and polyneuropathy. Generalized hypotonia, muscle weakness, and difficulty being weaned from the ventilator all point to the diagnosis. Neuromuscular blockade, prolonged ICU stay, steroids, and immobilization are all risk factors that resulted in the development of this condition in this patient. Treatment is with prolonged mechanical ventilation, extensive physical therapy, and discontinuing steroids.

Plasma exchange is an incorrect option because it is used to treat myasthenic crises. The absence of waxing and waning symptoms, ptosis, and fatigability make myasthenia unlikely.

Intravenous immunoglobulins is incorrect because it is the treatment of choice of Guillain-Barre syndrome (GBS), which manifests as ascending paralysis. It has no role in the management of critical illness myopathy and neuropathy.

Dantrolene is incorrect, it is used to treat neuroleptic malignant syndrome. In the absence of rigidity and fever, it is a less likely possibility.

Azathioprine is incorrect because it is the management of choice of autoimmune neuropathy due to vasculitis. There is no history of rash, joint pains, or purpuras to suggest a vasculitic disorder.

Free-running sleep disorder is correct. Free running sleep disorder (also known as non-24-hour sleep wake disorder or non-24) is one of the circadian rhythm sleep disorders in which the intrinsic circadian rhythm of the body is no longer entrained to the 24-hour light-dark cycle. People with free running sleep disorder manifest clinically with periodically recurring difficulty with sleep initiation, maintenance, and waking up. These individuals complain of insomnia and excessive daytime sleepiness due to abnormal synchronization between the normal sleep-wake schedule and their drifting sleep cycle. They also have a progressive delay in sleep onset time over nights and subsequently they sleep cycle gets disturbed over weeks or months. Free running sleep disorders are commonly associated with psychiatric disorders particularly depression, anxiety, panic disorder, and agoraphobia. Treatment of free-running sleep disorder includes melatonin and light therapy for entrainment and stabilization of the circadian rhythm.

Advanced phase sleep disorder is incorrect as it is related to the circadian rhythm and occurs at a fixed time. Advanced phase sleep disorder people (morning larks) also wake up very early and sleep many hours before normal people

Delayed phase sleep disorder is incorrect as it is related to the circadian rhythm and occurs at a fixed time. Delayed phase sleep disorder people (night owls) also sleep late and wake up late.

Jet lag sleep disorder is incorrect as it occurs due to rapid travel across time zones.

Shift-work sleep disorder is incorrect as it occurs in people who change their work shifts between light and dark frequently.

Cytomegalovirus is correct. The question describes an immunocompromised patient with a hematological malignancy on an immunosuppressive agent. Respiratory syncytial virus, influenza virus, parainfluenza virus, and adenovirus can also lead to pneumonia in both immunocompetent and immunocompromised patients. However, cytomegalovirus is the most commonly encountered virus in immunosuppressed individuals. Particularly at risk are those with HIV and/or hematological malignancies. It is estimated that the incidence of cytomegalovirus pneumonia in patients with hematological malignancies range from 2.3% to 16%. CMV and ammonia are associated with high mortality rates. The clinical presentation of cytomegalovirus infection varies from asymptomatic cases to severe pneumonia and disseminated viremia. Definitive diagnosis requires the presence of symptoms and signs of pneumonia and the detection of cytomegalovirus in lung tissue. PCR studies can confirm the diagnosis and determine the viral load. The mainstay of treatment of cytomegalovirus pneumonia is a combination of ganciclovir and cytomegalovirus immunoglobulin. Foscarnet and cidofovir are alternative options.

Pulmonary Langerhans cell histiocytosis is correct. Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon interstitial lung disease of especially young adults. Strong presence of CD1a antigen cells, is almost pathognomic of the disease on biopsy and >5% CD1a cells on bronchoalveolar lavage is also diagnostic.

Tuberculosis would be expected to present with typically more constitutional symptoms and a productive cough. A chest x-ray in this context would also be expected to demonstrate evidence of TB or old TB such as upper lobe fibrosis.

Pseudo-desquamative interstitial pneumonia is a distinct type of interstitial pneumonia and typically effects patients in their fourth or fifth decades of life. A chest X-ray or CT in this context would be expected to demonstrate ground glass opacities however a bronchoalveolar lavage would not be expected to demonstrate CD1a cells.

Respiratory bronchiolitis (RB), is a well-recognized pathological lesion found in the lungs of cigarette smokers and is not to be mixed with respiratory bronchiolitis-interstitial lung disease. RB would be expected to present with tan-pigmented macrophages (aka smokers macrophages) in the respiratory bronchioles and peribronchiolar fibrosis on imaging.

Idiopathic pulmonary fibrosis would be expected to present with a normal bronchoalveolar lavage and basilar, peripheral, bilateral, asymmetric, reticular opacities on chest x-ray.

PDE5 inhibitors is correct. This question describes a case wherein acute vasoreactivity testing was previously positive, but response to long-term calcium channel blockers therapy has proven unsustainable. Hypoxemia should always be treated. Subsequently, best treatment options for patients with lower risk would be endothelin receptor antagonists (ERAs) or phosphodiesterase 5 inhibitors (PDE5).

Warfarin is incorrect because it is an anticoagulant that is recommended for all patients with a diagnosis of idiopathic pulmonary arterial hypertension

Diuretics is incorrect as it should be used as a management option for possible heart failure.

Digoxin is incorrect as it has various inotropic effects.

Atrial septostomy is a treatment of choice when other treatments for pulmonary arterial hypertension (PAH) show to be ineffective.

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to aspergillus and other fungal species. A more inclusive name for this disease is Allergic Bronchopulmonary Mycosis (ABPM) and can be seen in patients with asthma or cystic fibrosis. ABPA/ABPM typically develop following the colonization of airways of patients with asthma or cystic fibrosis with Aspergillus and other spp. This colonization then leads to vigorous antibody and cell-mediated immune responses to mycosis antigens, hence asthma exacerbations and if left untreated ultimately fibrosis and bronchiectasis. Diagnosis is usually based on signs and symptoms of asthma, superimposed with a productive cough with a typically brown phlegm and fever. Other diagnostic clues in this patient are peripheral eosinophilia, the chest x-ray remarkable for bilateral upper lobe infiltrates and the fact that he has started working in a warehouse environment wherein he would have a potential greater chance of exposure to mold spores. The fact that the patient has elevated serum specific IgE levels against the recombinant Aspergillus allergen f4 also helps confirm the diagnosis as elevated levels of serum specific IgE in this context have 100% specificity and about 65% sensitivity for diagnosis of ABPA.

Asthma exacerbation can be differentiated by the infiltrates in the chest, the ongoing exacerbation and the brown phlegm.

Churg-Strauss syndrome can present with similar signs and symptoms of asthma exacerbation and would also be expected to present with sinusitis and other organ system involvement. The chest x-ray in this context would be expected to demonstrate transient, patchy opacities without lobar or segmental distribution.

Hypereosinophilic syndrome would be expected to mainly present with extrapulmonary involvement such as the heart, the GI tract or the brain on top of possible pulmonary involvement.

Chronic eosinophilic pneumonia would present with similar symptoms as ABPA and maybe related to asthma, but would be identified with pathognomic chest x-ray finding of bilateral peripheral or pleural-based infiltrates, described as photographic negative of pulmonary edema.

Administer levofloxacin is correct. It is reasonable to initiate the empiric antimicrobial treatment with fluoroquinolone taking into account the risks of comorbidities and bacterial resistance. Levofloxacin is best option among the given choices for highly suspected or established community-acquired pneumonia (CAP).

Hospitalize and take blood cultures is incorrect. It is generally advised to start the empiric treatment in an outpatient setting for patients with relatively moderate cases of community-acquired pneumonia (CAP). The decision of hospitalization is evaluated with either pneumonia severity index (PSI) or CURB-65 score and is generally indicated in the presence of serious comorbidities and other risk factors that might lead to failure of the treatment.

Administer azithromycin is incorrect. Macrolides are a first-line treatment option in most of the cases of community-acquired pneumonia (CAP), but used in combination with a beta lactam and not alone.

Administer amoxicillin is incorrect. This medication is usually used in a combination with macrolides as a first-line treatment. However, its effectiveness is lower when used alone rather than in combination, and in general amoxicillin with clavulanate is preferred over amoxicillin alone.

Perform bronchoscopy is incorrect. Bronchoscopic evaluation with either bronchoalveolar lavage or protected specimen brushing is indicated in severe cases of pneumonia, which are characterized with a more serious clinical presentation.

Strawberry gingival hyperplasia is pathognomonic of granulomatosis with polyangiitis (GPA). Ear, nose, and throat involvement is present in up to 99% of cases of GPA and may include chronic rhinitis and/or sinusitis, sinus pain, epistaxis, and nasal crusting. Destruction of the nasal cartilage can lead to nasal septal perforation or the saddle-nose deformity. Other manifestations include ulcerations of the oropharynx, gingival hyperplasia, and the rare strawberry gingival hyperplasia that is pathognomonic of GPA. While gingival hyperplasia is not necessarily uncommon in other disease processes, the ‘strawberry’ lesion is characterized by a granular, erythematous, and hemorrhagic appearance of the gingiva, resembling a “ripe strawberry,” and is a characteristic mucocutaneous finding of GPA.

Leukocytoclastic vasculitis is a histopathologic term used to describe small-vessel vasculitis. It classically presents as palpable purpura and is the most common dermatologic manifestation in GPA.

Koplik spots are clustered white lesions on the buccal mucosa that are pathognomonic of measles.

Mononeuritis multiplex is incorrect. Patients with GPA may have mononeuritis multiplex, typically a sensorimotor polyneuropathy with asymmetric involvement (i.e., foot or wrist drop). However, these findings are not pathognomonic of GPA.

Erythema chronicum migrans refers to the rash often seen in the early stage of Lyme disease and is pathognomonic for it.

RV strain is correct as it is strong evidence of right heart problems.

Left axis deviation is incorrect because ECG changes related to pulmonary hypertension are: right axis deviation, signs of right atrial enlargement, signs of right ventricular hypertrophy, and T wave inversion.

Signs of left atrial enlargement is incorrect because ECG changes related to pulmonary hypertension are: right axis deviation, signs of right atrial enlargement, signs of right ventricular hypertrophy, and T wave inversion.

Signs of left ventricular hypertrophy is incorrect because ECG changes related to pulmonary hypertension are: right axis deviation, signs of right atrial enlargement, signs of right ventricular hypertrophy, and T wave inversion.

T wave peaking is incorrect because ECG changes related to pulmonary hypertension are: right axis deviation, signs of right atrial enlargement, signs of right ventricular hypertrophy, and T wave inversion.

Elevated serum vascular endothelial growth factor D is correct as lymphangioleiomyomatosis (LAM) cells express this protein such that increased levels are diagnostically supportive of LAM.

Thin walled cysts on high resolution CT scan is incorrect because they may also be seen in pulmonary Langerhan’s cell histiocytosis and emphysema and are, therefore, less specific.

Restrictive features on pulmonary function tests is incorrect because pulmonary function testing in lymphangioleiomyomatosis (LAM) patients may reveal not only restrictive patterns, but also obstructive or mixed patterns. Therefore, these tests would not be conclusive.

Increased white cell count in bronchoalveolar lavage fluid is incorrect because studies have not found this to be a specific feature in these patients.

Raised erythrocyte sedimentation rate and C reactive protein is incorrect because while their levels may be raised, this occurs in several lung diseases as well as other diseases, and is not specific to lymphangioleiomyomatosis.

Decreased catheter related bloodstream infections by 66% in the first 3 months is correct. The Keystone initiative is a quality improvement initiative that aimed to decrease catheter-related bloodstream infections (CR-BSI) in the ICU. The results of the initiative were published in 2008. It included 108 ICUs, and the study duration was 18 months, in which rigorous and strict measures were taken, all evidence based, in order to limit the rate of CR-BSI. These measures included handwashing, using full barrier precautions during the insertion of central venous catheters, cleaning the skin with chlorhexidine, avoiding the femoral site if possible, and removing unnecessary catheters. These 5 measures are relatively simple, and have a high potential of improving outcomes, yet they are only applied fully in 30% of the times. In order to ensure that the medical and nursing teams apply these 5 measures fully, a checklist was put in effect. In the first three months of the project, the infection rate in Michigan’s ICUs decreased by 66%. The typical ICU – including the ones at Sinai-Grace Hospital – cut its quarterly infection rate to zero. Michigan’s infection rates fell so low that its average ICU outperformed 90% of ICUs nationwide. In the Keystone Initiative’s first 18 months, the hospitals saved an estimated $175 million dollars in costs and more than fifteen hundred lives, and the results sustained for almost four years.

More than 25% eosinophils in bronchoalveolar lavage fluid is correct as this is one of the diagnostic criteria for acute eosinophilic pneumonia, and the rise of eosinophils is significant in this fluid.The patient’s history and age are consistent with a diagnosis of acute eosinophilic pneumonia (AEP).

The duration of symptoms is about two months is incorrect because according to the diagnostic criteria for acute eosinophilic pneumonia (AEP), acute illness should be less than or equal to one month in duration.

Presence of eosinophilia in peripheral blood is incorrect because eosinophilia isn’t remarkable initially in acute eosinophilic pneumonia (AEP); even if present, it may be attributable to other fungal or parasitic causes.

Bilateral localized opacifications on lung CT is incorrect because the opacities in acute eosinophilic pneumonia (AEP) tend to be diffuse and scattered, rather than localized termed “the photographic negative of pulmonary edema”.

Suspicion of bronchopulmonary aspergillosis is incorrect because this would be a reason to exclude a diagnosis of acute eosinophilic pneumonia.

Corticosteroids use is correct. Late Steroid Rescue Study (LaSRS) showed that the use of corticosteroids in ARDS after 14 days of onset was found to increase mortality, while the use between 7 and 13 days tended to decrease mortality. The secondary outcomes found by this study concluded that the use of corticosteroids lead to fewer episodes of pneumonia and days of circulatory shock. However, this intervention was also associated with more adverse effects, such as neuromyopathy. The study also concluded that the use of corticosteroids in ARDS patients lead to a reduced duration of mechanical ventilation, but not in ICU days or total hospital length of stay. The final recommendation by LaSRS in regards to the use of corticosteroids was to follow a high dose and short course steroids to prevent ARDS or improve outcome in early established ARDS, the use of methylprednisolone was not recommended for ARDS of >14 days duration.

Right heart catheterization is correct as this patient likely has idiopathic pulmonary arterial hypertension (IPAH). Right heart catheterization is the gold standard for the diagnosis of pulmonary hypertension. Pulmonary hypertension is defined as the pulmonary arterial pressure of >20 mm Hg.

CT scan of the chest is not indicated as it does not provide a pressure measurement required for the diagnosis. However, a CT scan can be used to exclude mediastinal disease or interstitial lung disease with a high-resolution CT scan.

Ventilation/perfusion scan (V/Q) is useful for the diagnosis of chronic thromboembolic disease. In idiopathic pulmonary arterial hypertension (IPAH), V/Q scanning shows a heterogeneous or mottled perfusion pattern, while anatomic perfusion defects of the segmental or lobar level are more likely to represent thromboembolic disease.

Transthoracic echocardiogram (TTE) is a noninvasive method that can measure pulmonary artery systolic pressure (PASP). A PASP >40 mm Hg is suggestive of PH but is not, in all cases, diagnostic.

Pulmonary function testing (PFTs) is not diagnostic of pulmonary arterial hypertension (PAH) but can be used to identify obstructive or restrictive lung diseases.

Nebulize with albuterol would be the best treatment for his symptoms of hypoxia, most probably due to an exacerbation of asthma. It is given as 2.5 mg by continuous flow (updraft) nebulization every 20 minutes until improvement or signs of toxicity develop. Drying of respiratory secretions will be avoided unlike with the use of an anti-muscarinic agent like ipratropium.

Nebulize with ipratropium is incorrect. It won’t prevent the drying of respiratory secretions as it’s an anti-muscarinic agent.

Helium oxygen mixture is not first-line therapy, but the low density of helium can be used to provide oxygen in restrictive air spaces. It can be used in severe exacerbation.

IV beta adrenergic blocker will worsen the bronchospasm by inhibiting the beta receptors. >br>
Racemic epinephrine is indicated in refractory bronchospasm in the setting of anaphylaxis. It should be avoided in patients with known or suspected coronary artery disease.

Alpha 1 antitrypsin deficiency is a familial disease that affects both liver and lungs. The patient has a history of elevated liver enzymes and has severe emphysema. The patient may present with symptoms related to both or only one organ. The patient should strictly avoid smoking and areas with air pollution. There is also family history as the patient’s brother had passed away with similar symptoms. Bronchodilators can help alleviate his symptoms. Vaccinations can improve the patient’s quality of life.

Primary hemochromatosis is an autosomal recessive disease that leaves iron deposits in the heart, liver, and pancreas.

Wilson disease is an autosomal recessive condition with a disorder of copper metabolism.

Idiopathic pulmonary hemosiderosis is incorrect. There is no pulmonary involvement. It usually manifests with neurological symptoms and the classic K-F ring in the eye.

Primary sclerosing cholangitis manifests in patients with ulcerative colitis. Hemosiderosis is focal deposition of hemosiderin that does not involve tissue damage.

Pleural plaques usually develop more than ten years after asbestos exposure is correct. Asbestos-associated lung disease includes disorders of the lung and pleura caused by prolonged inhalation of asbestos fibers, such as asbestosis, diffuse pleural thickening, pleural plaques, pleural effusion, round atelectasis, diffuse malignant mesothelioma, and lung cancer. In order to make a diagnosis of asbestos-associated lung disease, the patient should have a history of exposure to asbestos fibers and a suitable latency period before the development of symptoms or radiographic findings. Pleural effusions may be the earliest clinical phenomenon, occurring as early as one year after asbestos exposure, whereas pleural plaques may require a latency period of more than ten years. Asbestosis, lung cancer, and diffuse malignant mesothelioma usually develop no sooner than twenty years after first and heavy asbestos exposure.

Asbestos fibers can only damage pleura but have no effect on lung parenchyma is incorrect. Asbestos fibers can damage both the lung parenchyma as well as the pleura, causing both benign and malignant disease.

Atelectasis does not usually develop in asbestos associated lung disease is incorrect. Pleural thickening occurring as a result of asbestos exposure may entrap a section of the lung, causing atelectasis and associated volume loss.

Asbestos associated lung disease has no significant association with lung cancer is incorrect. Asbestos has been found to be carcinogenic to humans and is clearly associated with increased lung cancer risk.

Smoking cessation has not been found to be beneficial in patients with asbestos exposure is incorrect. Tobacco smoking additively, and possibly synergistically, increases lung cancer risk in persons who have even short-term exposure to asbestos. Therefore, tobacco cessation is imperative in patients with asbestos exposure.

Mild relapse of microscopic polyangiitis (MPA) is often managed with increases in the dosage of oral steroids is correct. As in granulomatosis with polyangiitis (GPA), remission induction in microscopic polyangiitis (MPA) includes oral steroids along with cyclophosphamide. However, relapses are common in MPA. Many patients with MPA may experience disease relapse during the tapering of remission maintenance or after the therapy is discontinued. Relapses, however, are generally milder than the initial presentation, though some may include end-organ damage.

Remission induction of MPA includes cyclophosphamide only is incorrect. As in granulomatosis with polyangiitis (GPA), remission induction in microscopic polyangiitis (MPA) includes oral steroids along with cyclophosphamide.

Remission induction of MPA includes plasma exchange and oral steroids is incorrect. Mild relapse is often managed by increasing the dosage of oral steroids, whereas major relapse often requires the reintroduction of initial therapy. Plasma exchange is not included in the remission induction in MPA but may be considered in case of treatment failure.

Cyclophosphamide is effective in preventing disease relapse is incorrect. Cyclophosphamide is effective for the treatment of active disease; however, it has not been shown to prevent disease relapse in MPA.

Rituximab cannot be used in patients with MPA is incorrect. Rituximab may be used in patients with MPA who are intolerant to cyclophosphamide.