Pulmonary/CC Board Review

A 25-year-old woman is evaluated for recurrent episodes of acute dyspnea associated with rapid breathing, tightness in her chest and throat, voice changes, and inspiratory difficulty. When she has these episodes she feels that she “can’t get enough air.” The symptoms typically start and resolve abruptly, and treatment with albuterol provides only minimal relief. On physical examination, her vital signs are normal. Lungs are clear. Spirometry shows an FEV1 of 2.8 L (88% of predicted) and FEV1/FVC ratio of 80%. Which of the following is the most appropriate next diagnostic step?

1. Chest radiography2. CT scan of the neck3. Flow volume loops4. Thyroid function tests

Vocal Cord Dysfunction

A 35-year-old man is evaluated for episodic wheezing, dyspnea, and cough. Two months ago he had an acute episode of cough, dyspnea, wheezing, and chest tightness within minutes of inhaling high concentrations of ammonia solution after an accidental spill at work. In the emergency department, the chest radiograph was normal, and he received inhaled bronchodilators and a brief course of oral corticosteroids. The patient does not have a history of asthma or allergies and is otherwise healthy and takes no medications. The physical examination is normal. Spirometry shows an FEV1 of 90% of predicted; FEV1/FVC ratio is 82%. Which of the following is the most appropriate next test in this patient’s evaluation?

1. Bronchoscopy with biopsy2. Inhalation challenge with chlorine3. Methacholine challenge test4. Spirometry before and after work

Asthma- Diagnosis

• Spiromery shows low FEV1/FVC ratio and reversibility 12% of greater improvement in FEV1 after administration of bronchodilators

• Next step bronchial challenge test

A 27-year-old man is evaluated for a 6-month history of cough, which is worse at night and after exposure to cold air. Often his cough is brought on by taking a deep breath or by laughter. He does not have postnasal drip, wheezing, or heartburn. He has a strong family history of allergies. Physical examination, chest radiograph, and spirometry are normal. He receives no benefit from a 3-month trial of gastric acid suppression therapy, intranasal corticosteroids, and an antihistamine-decongestant combination. Which of the following would likely provide the diagnosis of this patient’s chronic cough?

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1. 24-Hour esophageal pH monitoring2. Bronchoscopy3. CT scan of the chest4. CT scan of the sinuses5. Trial of inhaled albuterol

Vocal Cord Dysfunction

Aspirin-Sensitive Asthma

Exercise-Induced Bronchospasm

ABPA

GERD and Asthma

Cough-Variant Asthma

Virus-Induced Asthma

Reactive Airways Dysfunction Syndrome

Occupational Asthma

A 28-year-old man is evaluated for a 6-month history of episodic dyspnea, cough, and wheezing. As a child, he had asthma and allergies, but he has been asymptomatic since his early teenage years. His recent symptoms started after an upper respiratory tract infection, and they are often triggered by exercise or exposure to cold air. He is also awakened with asthma symptoms 5 or 6 nights a month. He is otherwise healthy and takes no medications.

On physical examination, vital signs are normal. There is scattered wheezing in both lung fields. Chest radiograph is normal. Spirometry shows an FEV1 of 70% of predicted with a 15% improvement after inhaled albuterol.

Which of the following is the most appropriate therapy for this patient?

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1. Azithromycin2. Inhaled Albuterol as needed3. Inhaled low-dose corticosteroids plus inhaled albuterol as needed4. Long-acting B-agonist5. Long-acting B- agonist plus inhaled albuterol as needed

PersistentComponents of Severity

Intermittent Mild Moderate Severe

ImpairmentSymptoms ≤2 days/week > 2 days/week Daily Throughout the day

Nighttime awakenings ≤2 ×/month 3-4 ×/month >1 ×/week but not nightly

Often 7 ×/week

SABA use for symptom control (not prevention of EIB)

≤2 days/week >2 days/week but not more than 1 ×/d

Daily Several times a day

Interference with normal activity

None Minor limitation Some limitation Extremely limited

Lung function Normal FEV1 between exacerbations FEV1 >80% of predicted FEV1/FVC normal

FEV1 >80% of predicted FEV1/FVC normal

FEV1 >60% but <80% of predicted FEV1/FVC reduced 5%

FEV1 <60% of predicted FEV1/FVC reduced >5%

RiskExacerbations (consider frequency and severity)b,c

0-2/year >2/year

Recommended step for initiating treatment (see Figure 4 for treatment steps)d

Step 1 Step 2 Step 3; consider short courses of systemic corticosteroids

Step 4 or 5; consider short courses of systemic corticosteroids

A 70-year-old woman is evaluated for a 6-month history of fatigue, an unintentional weight loss of 4.4 kg (10 lb), an increase in chronic cough with sputum production, and a decrease in exercise capacity. The patient has a 40-pack-year history of cigarette smoking but stopped smoking 10 years ago when chronic obstructive pulmonary disease was diagnosed. She has no other symptoms and specifically denies abdominal pain, nausea, vomiting, diarrhea, or change in her bowel habits. Her medications are albuterol as needed, an inhaled corticosteroid, and salmeterol. She has been on stable dosages of these drugs for 18 months. Age- and sex-appropriate cancer screening tests done 6 months ago were normal. On physical examination, the temperature is 37.5 °C (99.5 °F), the blood pressure is 128/76 mm Hg, the pulse rate is 94/min and regular, the respiration rate is 16/min, and the BMI is 20. Heart sounds are distant, and breath sounds are diminished bilaterally. There are no abdominal masses or organomegaly and no peripheral edema.Labs: Hg: 15, Alb: 3.0, Cr: 0.8, TSH: 2.0Spirometry shows an FEV1 of 40% of predicted and an FEV1/FVC ratio of 45%. Chest radiograph shows hyperinflation. Which of the following is the most likely reason for this patient’s weight loss?

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0% 0%0%0%1. Breast Cancer2. Cervical Cancer3. Colon Cancer4. Chronic Obstructive Pulmonary Disease

A 50-year-old man is evaluated for a 1-year history of cough productive of mucoid sputum. He has not had fever or lost weight during that time. The patient has smoked one pack of cigarettes a day for 20 years. He has no history of allergic disease or gastroesophageal reflux disease. On physical examination, the temperature is 37.0 °C (98.6 °F), the blood pressure is 124/76 mm Hg, the pulse rate is 78/min and regular, and the respiration rate is 15/min; BMI is 25. The lungs are clear, and the chest radiograph is normal. Spirometry shows an FEV1 of 85% of predicted and an FEV1/FVC ratio of 75%; there is no change in results after administration of a bronchodilator. Which of the following is the most likely diagnosis?

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1. Asthma2. At risk of chronic obstructive pulmonary disease (COPD)3. Mild (GOLD stage I) COPD4. Moderate (GOLD stage II) COPD

COPD

Table 8

At risk --- Normal SpirometryChronic Symptoms

GOLD 1 Mild FEV1/FVC <70%FEV >80% of predicted

GOLD 2 Moderate FEV1/FVC <70%50%<FEV1<80% of predicted

GOLD 3 Severe FEV1/FVC<70%30% < FEV1< 50% of predicted

GOLD 4 Very Severe FEV1/FVC <70%FEV1< 30% of predicted

All in patient with FEV1/FVC < 70%

A 55-year-old man with a 7-year history of severe chronic obstructive pulmonary disease is evaluated after being discharged from the hospital following an acute exacerbation; he has had three exacerbations over the previous 18 months. He is a long-term smoker who stopped smoking 1 year ago. He adheres to therapy with albuterol as needed and inhaled salmeterol and tiotropium and has demonstrated proper inhaler technique. On physical examination, vital signs are normal. Breath sounds are decreased bilaterally; there is no edema or cyanosis. Oxygen saturation after exertion is 92% on ambient air. Spirometry shows an FEV1 of 32% of predicted and an FEV1/FVC ratio of 40%. Chest radiograph done in the hospital 3 weeks ago showed no active disease. Which of the following should be added to this patient’s therapeutic regimen?

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1. An inhaled corticosteroid2. Ipratropium3. N-acetylcysteine4. Oral Prednisone

Level of Airflow Obstruction Recommended Therapy

FEV1 60-80% of predicted(THINK GOLD 1 or 2)

Inhaled bronchodilator therapy (short acting anticholinergic or short acting B2 agonist)

FEV1< 60% of predicted(THINK GOLD 3)

Daily monotherapy with an Inhaled bronchodilator therapy (long acting anticholinergic or long acting B2 agonist)

Consider combination inhaled therapy (long acting anticholinergic, long acting B2 agonist, or cortisteroid)

FEV1< 50% of predicted(THINK GOLD 3 and 4)

Consider adding pulmonary rehabilitation

A 64-year-old woman is evaluated for a 6-week history of dyspnea, dry cough, fever, chills, night sweats, and fatigue, which have not responded to treatment with azithromycin and levofloxacin; she has lost 2.2 kg (5 lb) during that time. The patient had a thorough examination 6 months ago while she was asymptomatic that included routine laboratory studies, age- and sex-appropriate cancer screening, and a chest radiograph; all results were normal. The patient has never smoked, has had no known environmental exposures, and has not traveled recently or been exposed to anyone with a similar illness. Her only medications are aspirin and a multivitamin. On physical examination, temperature is 37.8 °C (100.0 °F); other vital signs are normal. Cardiac examination is normal. There are scattered crackles in the mid-lung zones with associated rare expiratory wheezes. There is no digital clubbing. Musculoskeletal and skin examinations are normal. Chest radiograph is shown . Which of the following is the most likely diagnosis?

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0% 0%0%0%1. Cryptogenic organizing pneumonia2. Idiopathic pulmonary fibrosis3. Lyphocyti interstitial pneumonia4. Nonspecific interstitial pneumonia

55 year old F with fever, dyspnea, malaise for 2 weeks…

Cryptogenic Organizing Pneumonia• History: Insidious onset (weeks to 1-2 months), often failed

outpatient treatment for viral pneumonitis or bacterial pneumonia

• Epidemiology: M=F, Age=middle aged• HPI: Cough, fever, dyspnea, malaise, and myalgias• PE: Rales• Imaging: bronchial thickening, some interstitial findings

patchy bilateral alveolar infiltrates, MIGRATES• Histology: organizing pneumonia, bronchiolitis and chronic

alveolitis (open lung bx preferred, often adequate with TBB)• Associations: Penicillamines and RA• Treatment: Good prognosis, responds to steroids. Slow taper

to avoid exacerbations

55 year old male presents with 6 months dyspnea, cough…

Usual Interstitial pneumonia

Idiopathic Pulmonary Fibrosis (IPF)• History: VERY slow onset, at least 6 months• Epidemiology: M=F, average age = 55• HPI: dyspnea, poor exercise tolerance, cough, NO FEVER• PE: no extrapulmonary manifestations, CLUBBING usually seen, coarse,

dry crackles at lung bases• Imaging: CT “ground glass” early, “honeycombing “late, CXR interstitial• Histology: Usual Interstitial Pneumonia (UIP)• Associations: smoking exacerbates, ~ 10% low titers of ANA or RF, no

associations with infections, drugs, chemicals• Treatment: steroids +/- cyclophosphamide or azathiprine (20-30%

improve), Single-lung transplant in late cases• Response: A-a gradient response to exercise.

45 year old F, h/o IVDA, with dyspnea and cough over last 2-3 months…

Nonspecific Interstitial Pneumonitis (NSIP)

• History: subacute presentation 2-3 months coughing, dyspnea• PE: bibasilar crackles, stimata of CTD• Imaging: basilar or mid-lung reticular changes, (spares subpleural region)

rarely with honeycombing, pattern of ground-glass opacification in the mid- and lower-lung zones

• Diagnosis: requires open lung biopsy (difficult to differentiate fro IPF)• Histo: lymphoplasmacytic interstitial infiltration in a uniform pattern,

accompanied by chronic fibrosis (NSIP)• Associations: HIV, CTD, HSP, drug induced lung disease • Treatment: steroids +/- cytotoxic meds• Prognosis: better that IPF, but still grim when fibrosis on bx

38 year old F presents with acute onset shortness of breath…

Lymphangioleiomyomatosis• Epidemiology: Premenopausal women• Pathophys: Immature smooth muscle proliferation in lymph, vasc, and

alveolar wall/perbronchial structures cysts and constriction in these structures

• HPI: often presents with pneumothorax• PE: hamartomas, ash leaf spots, adenoma sebaceum• Imaging: honeycombing diffusely throughout the lung (in contrast to

upper lung in Langerhans)• Associations: Pneumothorax, chylous pleural effusions (TG >110 +/-

chlomicrons, common with Tuberous Sclerosis or multiple hamartomas (Cowden’s Syndrome)

• Treatment: Oophorectomy with progestin treatment, lung transplant (may recur)

32 year M, smoker, presents with gradual worsening of shortness of breath/cough…

Pulmonary Langerhans Cell Histocytosis

• History: 10% present with pneumothorax, 50% will have PTX sometime during illness

• Epidemiology: M>F, smokers, majority <40 y/o• PFTs: OBSTRUCTIVE• Imaging: interstitial changes and small cystic spaces in upper lung fields,

honeycomb, • Histology: Langerhans cells• Associations: smoking• Treatment: stop smoking, steroids generally do not help. Occasional

spontaneous resolution• Hand-Schuller Christian syndrome: lytic bone lesions, diabetes insipidius,

exophthalmus• Smoking related ILDs: Pulm Langerhans, Resp Bronchiolitis, DSIP

60 year old F with h/o asthma presents with 3 months insidious cough, wheezing, night sweats, low grade fevers…

ESR: 82

BAL: high percentage eosinophils

Eosinophilic Pneumonias

• Acute, benign eosinophilic pneumonia: Loefflers syndrome, minimal resp symptoms. MIGRATORY infiltrates. Peripheral eosinophilia. Rule out drugs and parasites.

• Acute eosinophilic pneumonia: acute, febile, hypoxemic resp failure resembling ARDS. Steroids and vent support.

• Chronic Eosinophilic pneumonia: most common in U.S. Middle aged women. Subacute. CXR bilateral, very peripheral infiltrates, photographic negative of pulm edema, High ESR. Steroids. Relapse common.

55 year old f presents with joint pain, Rash & fevers…

Sarcoidosis• Lofgren’s sndrome: Erythema nodosum

(good prognosis), polyarthralgias, hilar LAD, and fever

• Multisystem disease• Imaging: bilateral hilar and/or mediastinal

adenopathy (disappears as dz progresses), +/- parenchymal infiltrates

• Histo: noncaseating granuloma• BAL: increased lymphs, helper/supressor

ratio of > 4:1 (opposite of HSP)• Associations: ACE level, hypercalcemia,

hypergammaglobulinemia• Treatment: 75 % recover without

treatment, steroids for severe cases

Stage Radiographic Pattern

0 Normal

I Hilar lymphadenopathy with normal parenchyma

II Hilar lymphadenopathy with abnormal parenchyma

III No lymphadenopathy with abnormal lung parenchyma

IV Parenchymal chagnes with fibrosis and architectural distortion

Histology Clinical Diagnosis

UIP IPF/ Cryptogenic fibrosing alveolitisCTD associated lung diseaseRadiation

NSIP NSIP (CTD, HIV, Drugs)

Organizing Pneumonia Cryptogenic Organizing Pneumonia

Respiratory Bronchiolitis Respiratory Bronchiolitis ILD -DSIP

Lymphoid Interstitial pneumonia Lymphoid Interstitial pneumonia

Diffuse Alveolar Damage Acute Interstitial Pneumonia

A 30-year-old medical resident is evaluated for cough, right-sided chest pain, and fever of 21 days’ duration. He has no significant medical history or family history, and he takes no medications. Hemoglobin is 14 g/dL (140 g/L), and the leukocyte count is 8000/µL (8 × 109/L). Chest radiograph shows a right pleural effusion occupying approximately 50% of the hemithorax without other abnormalities. Thoracentesis yields turbid, yellow fluid, and analysis shows:

RBC Count: 500 uL, Nucleated Cell count: 3500 with 20% neutrophils, 60%lymphocytes, 10% macrophages, 4%mesothelial cell, and 6% eosinophilsTotal Protein: 4.2, LDH: 240, pH: 7.35, Glucose: 68Serum total protein is 7.0 g/dL and serum lactate dehydrogrenase is 100U/L. Gram stain shows no organism and culture is pending.

Which of the following is the most appropriate next step in management?

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1. Chest CT scan2. Flexible bronchoscopy3. Pleural biopsy4. Repeat chest radiograph after a 5-day course of azithromycin

A 70-year-old man is evaluated in the emergency department for a 2-day history of dyspnea with exertion, orthopnea, and paroxysmal nocturnal dyspnea. He has ischemic heart disease with left ventricular dysfunction and had coronary artery bypass graft surgery 6 weeks ago. His medications include aspirin, nitroglycerin, metoprolol, lisinopril, and furosemide. On physical examination, the patient is sitting upright and breathing with difficulty; the temperature is 37 °C (98.6 °F), the blood pressure is 150/85 mm Hg, the pulse rate is 105/min and regular, and the respiration rate is 28/min. Oxygen saturation is 89% on ambient air. There are fine crackles at the lung bases bilaterally, and breath sounds are diminished at the right base. There is a regular tachycardia and an S3 at the apex. There is no jugular venous distention or peripheral edema. Hemoglobin is 12.5 g/dL (125 g/L), and the leukocyte count is 10,500/µL (10.5 × 109/L). Chest radiograph shows cardiomegaly and small bilateral pleural effusions, greater on the right than the left. Thoracentesis is performed, and pleural fluid analysis shows:

Nucleated Cell Count: 450/UL with 3% neutrophils, 70% lymphocytes, 10% macrophages, 15% mesothelial cells, and 2% eosinophilsPleural Fluid to serum total protein ratio: 0.54, Plerual fluid to upper limits of normal serum LDH ration: 0.52, Glucose: 80, Total Protein: 3.7, pH: 7.45, Albumin: 1.5, Cholestrol 35, The serum-pleural fluid albumin gradient is 1.7.

Which is the most likely diagnosis?

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0% 0%0%0%1. Heart Failure2. Parapneumonic Effusion3. Post-cardiac injury syndrome4. Pulmonary embolism

Pleural Fluid Pearls

• WBC>1000 (exduate), >10,000 (parapneumonic effusion); >100,000 (pus/empyema)

• Eosinophils>10% (pneumothorax, drug reaction, parasites, fungus, asbestos)

• Lymphocytes>50% (TB, or cancer)• Glucose ~80 (TB); ~60 (cancer or empyema); <30 (RA)• Amylase (pancreatic fistula, esophageal rupture, local

malignancy)• pH<7.0 (loculated effusion/empyema), <7.2 (PLACE CHEST

TUBE), < 7.3 (with malignancy, suggests high tumor burder)• TG>115= chylous; <50= pseudochylous

A 64-year-old woman who resides in a nursing home and has a history of end-stage kidney disease for which she receives hemodialysis presents with fever, copious sputum production, and hypoxemia. Chest radiograph shows a right-sided infiltrate. She is intubated and started on vancomycin, levofloxacin, and piperacillin-tazobactam for treatment of diffuse right-sided pneumonia. On day 3 in the hospital, she has been afebrile for 48 hours, and her secretions are scant and white. On physical examination, the temperature is 36.9 °C (98.5 °F), pulse rate is 88/min, respiration rate is 14/min, and blood pressure is 140/86 mm Hg; oxygen saturation is 96% on FiO2 0.4. There are diffuse right-sided inspiratory crackles. Leukocyte count is 9600/µL (9.6 × 109/L) (20,400/µL [20.4 × 109/L] on admission). Admission sputum culture is now growing methicillin-resistant Staphylococcus aureus; blood cultures are negative. Chest radiograph shows slight improvement in the right-sided infiltrate compared with admission. Which of the following is the most appropriate antibiotic therapy at this time?

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1. Complete an 8-day course of all three antibiotics2. Complete an 8-day course of vancomycin and discontinue the other antibiotics3. Complete a 14-day course of all three antibiotics4. Complete a 14-day course of vancomycin and discontinue the other antibiotics5. Discontinue all antibiotics

A 30-year-old woman develops bilateral pulmonary infiltrates and hypoxemia 48 hours after undergoing repair of multiple long-bone fractures. Her initial arterial blood gases are pH 7.48, PCO2 30 mm Hg, and PO2 45 mm Hg on 100% oxygen by nonrebreather mask. The patient is intubated and placed on mechanical ventilation. The temperature is 38.3 °C (101.0 °F), the pulse rate is 100/min, the respiration rate is 28/min, and the blood pressure is 120/60 mm Hg. The patient weighs 60 kg (132.3 lb). Oxygen saturation is 83%. She is sedated, hemodynamically normal, and not using accessory muscles to breathe. There are bilateral inspiratory crackles. The ventilator settings are as follows: volume control mode, respiration rate 26/min, tidal volume 360 mL, positive end-expiratory pressure (PEEP) 5 cm H2O, FiO2 0.8. Arterial blood gases are pH 7.45, PCO2 33 mm Hg, and PO2 50 mm Hg. Which of the following is the most appropriate next step in the management of this patient?

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1. Increase FiO2 to 1.0

2. Increase PEEP to 10 cm H2O

3. Increase respiration rate to 32/min4. Increase tidal volume to 700 mL5. Start vecuronium infusion

ALI/ARDS

• ALI– PaO2/FiO2< 300– Bilateral infiltrates– PCWP <18mg Hg

• ARDS– PaO2/FiO2<200– Bilateral infiltrates– PCWP < 18 mg Hg

ARDS