Pulmonary Board Review

1. Pulmonary Board Review Questions from MKSAP and Harrison’s Self-Assessment

2. MKSAP Q15 • A 56 yoM evaluated for hemoptysis x 7 days & a recent 5-kg (11-lb) weight loss. He smoked 1 ppd x 40 yrs but stopped 3 wks ago. CXR shows a 4-cm irregular mass in the RUL. Fiberoptic bronchoscopy shows a friable tumor in the proximal RUL & biopsy reveals squamous cell CAR. A CT scan shows a 2.0-cm R paratracheal node in addition to the primary lesion. • Which of the following is the most appropriate next step in the patient's management? • A Chemotherapy • B Positron emission tomography (PET) • C Pneumonectomy • D Pulmonary function tests

3. In pts w/ potentially operable non-small-cell lung CA, PFTs are indicated to assess pulmonary reserve.

4. MKSAP Q50 • A 55 yoF w/ long-standing moderate to severe asthma is evaluated for worsening productive cough, dyspnea, and wheezing. She has no fever, chills, or CP. On exam, she is in NAD; her temp is 37 °C, BP is 110/70, HR 84, and RR 18. Chest exam reveals bilateral wheezing and scattered rhonchi over the upper lung fields. Cardiac exam is NL. CXR shows patchy infiltrates in both upper lobes and prominent bronchial markings c/w bronchiectasis, a finding confirmed on HRCT. The WBC is 8.5 with 45% neutrophils, 35% lymphocytes, 10% eosinophils, and 10% mononuclear cells. • Which of the following is the most appropriate next step in the evaluation of this patient? • A Bronchoscopy with lung biopsy • B Positron-emission tomographic scan • C Allergy skin testing • D Methacholine challenge test

5. ABPA • Characteristic features = moderate to severe persistent asthma, bronchiectasis and CXR abnls, elevated serum IgE, eosinophilia, & a positive skin test to Aspergillus fumigatus. • A skin test to determine the presence of allergic response to Aspergillus is an important 1st step in evaluating these pts, b/c ~all w/ ABPA have a (+) skin test. • However, many w/ (+) skin tests do not have ABPA; therefore, the test has a low PPV and high NPV. • Tx often requires systemic corticosteroids & itraconazole.

6. MKSAP Q80 • A previously healthy 35 yoM is evaluated for episodic wheezing, dyspnea, and cough. 2 mos ago he had an acute episode of cough, dyspnea, wheezing, and chest tightness within minutes of inhaling high concentrations of chlorine gas after an accidental spill at work. At that time he was seen in the ED; CXR was NL and he received a brief course of ABXs and po steroids. He has no prior h/o asthma or allergies and is o/w healthy. His exam is NL. Spirometry shows an FEV1 of 90% predicted; FEV1/FVC of 82%. • Which of the following is the most appropriate next test in this patient's evaluation? • A Bronchoscopy with endobronchial biopsy • B Methacholine challenge test • C Inhalation challenge with increasing concentrations of chlorine • D Spirometry before and after work shift

7. Reactive Airways Dysfnx Syndrome • Follows a single, accidental inhalation of high levels of a nonspecific respiratory irritant in pts who typically do not have a h/o asthma. • W/in minutes of exposure, the pt develops cough, wheezing, dyspnea, and chest tightness. Sxs persist even after exposure has stopped and may last for years, but can also resolve in a few months. • Dx is based on hxand confirmed by a (+) methacholinechallenge. • Other options • Exposure challenge with chlorine poses unnecessary risk of severe attacks. • Bronchial bx is not needed for the dx. • Spirometry before and after work shift is not helpful in cases of accidental exposure to irritants b/c these exposures are not likely to happen on a daily basis and therefore these measurements (which are used in other forms of occupational asthma) are not useful in the dx of RADS.

8. MKSAP Q93 • A 44 yoM is evaluated for nonproductive cough and progressive dyspnea. He is s/p a single-lung transplant last year for idiopathic pulmonary fibrosis. 2 mos post-transplant he had an episode of acute rejection successfully managed w/ augmented immunosuppression. 4 months later, a 2nd episode of acute rejection occurred, requiring another increase in immunosuppressive tx. Lung exam at this time shows early inspiratory crackles and PFT results are as follows: • FVC = 3.14 L (63% of expected) • FEV1 = 1.19 L (32% of expected) • FEV1/FVC = 0.38 (51% of expected) • CXR is NL. HRCT shows lobular hyperlucency and bronchodilatation. • Which of the following is the most likely diagnosis? • A CMV pneumonia • B Acute organ rejection • C Bronchiectasis • D PCP pneumonia • E Bronchiolitisobliterans

9. Transplant Assoc’d Bronchiolitis Obliterans • This pt has the typical findings of bronchiolitis obliterans associated with lung transplant, including cough, dyspnea, early inspiratory crackles, and severe airflow obstruction. • CXR is usually NL or may show hyperinflation. HRCT shows central lobular hyperlucency and bronchodilatation. • Occurs in 20-50% of long-term transplant survivors, and has a high mortality. It accounts for 30% of all deaths that occur after post-op year 3. • Probably represents a form of chronic rejection. Frequent and severe acute rejection episodes are the main risk factors.

10. MKSAP Q 123 • A 45 yoM is evaluated for mild DOE. He has smoked 1.5 ppd x 30 years. PMH and FH are unremarkable. The heart and lung exams are unremarkable and CXR is NL. Spirometry shows FEV1 of 70%, FVC of 75%, FEV1/FVC of 70%. After use of a bronchodilator, FEV1 increases to 80% and FVC to 85%; the FEV1/FVC ratio is 75%. Serum IgE is NL, and there are no eosinophils on peripheral smear. • Which of the following is the most likely diagnosis? • A Chronic obstructive pulmonary disease, stage 0 • B Chronic obstructive pulmonary disease, stage 1 • C Moderate persistent asthma • D Restrictive lung disease

11. Steps in PFT Interpretation • 1 - Acceptable & reproducible? Appropriate reference values? • 2 - If FEV1 & FEF 25-75% are > 80% predicted, spirometry is NL. • 3 - If FEV1 is < 80%, there could be an obstruction or restriction. • 3 – If FEV1/FVC is < 70% obstruction is present and bronchodilator inhalation is needed to check for reversiblility. • 4 – If FEV1/FVC is NL, restriction is present & lung volumes may be needed to better dx the restrictive dz. • 5 – Are obstruction & restriction both present? Is there a concomitant decrease in TLC &FEV1/FVC? • 6 – If FEF 25-75% is dec’d but FEV1 & FEV1/FVC are borderline NL, early obstruction or small airway dz is likely present.

12. GOLD Staging of COPD * Classification based on post-bronchodilator FEV1

13. Harrison’s Self Assessment • A 60 yoM seen in the clinic for counseling about asbestos exposure. He is well and has no Sxs. He smokes 1 ppd but has no other habits. He is currently retired but worked for 30 years as a pipefitter and says he was around "lots" of asbestos, often w/o wearing a mask or other protective devices. Exam is NL except for nicotine stains on the L 2nd and 3rd fingers. CXR shows pleural plaques but no other changes. PFTs, including lung volumes, are NL. • Which of the following statements should be made to this patient? • A. He must quit smoking immediately as his risk of emphysema is higher than that of other smokers because of asbestos exposure. • B. He does not have asbestosis. • C. His risk of mesothelioma is higher than that of other patients with asbestos exposure because he has a history of tobacco use. • D. He has no evidence of asbestos exposure on chest radiography. • E. He should undergo biannual chest radiography screening for lung cancer.

14. Asbestos and Asbestosis • Asbestos • Previously commonly used for insulation from the 1940s to the mid-1970s • Pulmonary manifestations include pleural plaques (calcifications/thickening along the parietal pleura), benign asbestos pleural effusions (often blood, can regress or progress spontaneously), asbestosis, lung CA, and mesothelioma. • Asbestosis • Refers to interstitial lung dz, generally w/ fibrosis, seen in the lower lung fields of a CXR or CT and an associated restrictive ventilatory defect. • This pt does not have interstitial changes on CXR and has no restriction on PFTs. Therefore, he does not have asbestosis. • Other answers • The risk of lung CA, including squamous cell and adenoCAR, is inc’d in all w/ asbestos exposure & even more so w/ smoking. However, mesothelioma risk, though elevated in patients with asbestos exposure, is not increased by smoking. • Despite the high risk of CAs in this group of pts, no benefit has been found w/ screening techniques, including biannual CXRschest radiograms.

15. Harrison’s Self-Assessment • A 45 yoM is evaluated in clinic for asthma. Sxsbegan 2 yrs ago and are characterized by an episodic cough and wheezing that responded initially to inhaled bronchodilators and inhaled corticosteroids but now require nearly constant Prednisone tapers. Sxsare worst on weekdays but he cannot pinpoint specific triggers. His medications are AlbuterolMDI, FluticasoneMDI, and Prednisone 10 mg PO daily. He has no habits and works as a textile worker. Exam is notable for mild diffuse polyphonic expiratory wheezing but no other abnormality. • Which of the following is the most appropriate next step? • A. Exercise physiology testing • B. Measurement of FEV1 before and after work • C. Methacholine challenge testing • D. Skin testing for allergies • E. Sputum culture for Aspergillusfumigatus

16. This pt p/w typical asthma Sxs; however, his Sxs are escalating and now require nearly constant use of oral steroids. • It is important to note that his Sxs are worse during weekdays and better on weekends. This suggests that there is an exposure during the week that may be triggering his asthma. • Often textile workers have asthma resulting from inhalation of particles. • The 1st step in dx’g a work-related asthma trigger is to check FEV1 before and after the 1st shift of the workweek. A decrease in FEV1 suggests an occupational exposure.

17. Harrison’s Self-Assessment • A 71 yoM p/w cough and sputum production. He occasionally coughs up a small amount of blood. His Sxshave worsened over a period of years, and he now gets winded going up 1 flight of stairs. He has a distant h/o of tx’d TB and has been treated for CAP 2-3x per year for the past several years. He received a flu vaccination this fall. He has never smoked. On exam, his RR is 16. He has scattered rhonchi and faint expiratory wheezes bilaterally. He is not using accessory muscles. You suspect that this patient may have bronchiectasis to explain his recurrent infections. • Which of the following is true regarding making this diagnosis? • A. Bronchiectasis cannot be diagnosed in the setting of an acute pulmonary infection. • B. Bronchoscopy is required to definitively diagnose bronchiectasis. • C. Chest x-ray demonstrating honeycombing pattern will make the diagnosis. • D. High-resolution chest CT scan is the preferred confirmatory test for bronchiectasis. • E. Physical examination is sufficient to diagnose bronchiectasis in a patient with this history.

18. Bronchiectasis • Abnl and permanent dilatation of bronchi. Can be focal or widespread. Generally affects older pts, F > M. Also inc’d in those w/CF and ciliarydysfnx. • Results from inflammation and destruction of the bronchial wall and is usually triggered by infection. Staph aureus, Klebsiella, viruses (eg, adeno and influenza) and mycobacteria (including TB) are the main causes. • Sxs = recurrent cough, purulent sputum, frequent lung infections. • Exam findings are variable and they, alone, are not sufficient for dx. • HRCT is the standard technique to confirm the dx of bronchiectasis, and it will often show the dilated airways beyond the central airways.

19. Harrison’s Self-Assessment • A 45 yoF p/w abnlsensations in her legs that keep her from sleeping at night. She 1st notices the Sxs ~8 pm when she is sitting quietly watching television. She describes the Sxsas "ants crawling in her veins." The Sxsare not painful, but they are very uncomfortable and worsen when she lies down at night. They interfere with her ability to fall asleep about four times weekly. If she gets out of bed to walk or rubs her legs, the Sxsdisappear almost immediately only to recur as soon as she is still. She also sometimes takes a very hot bath to alleviate the Sxs. During sleep, her husband complains that she kicks him throughout the night. She has no h/o neuroor renal dz. She is currently perimenopausaland has been having very heavy and prolonged menstrual cycles over the past several months. Her exam is NL. Her Hgb is 9.8 , MCV is 68, ferritin is 22. • Which is the most appropriate initial therapy for this patient? • A. Carbidopa/levodopa • B. Hormone replacement therapy • C. Iron supplementation • D. Oxycodone • E. Pramipexole

20. Restless Leg Syndrome • Affects 1–5% of young to middle-aged pts and as many as 20% of older pts. • Sxs = a nonspecific uncomfortable sensation in the legs that begin during periods of quiescence and are alleviated w/ movement. Patients frequently find it difficult to describe their Sxs, but usually describe the sensation as deep within the affected limb. Rarely is the sensation described as distinctly painful unless an underlying neuropathy is also present. • Severity tends to wax and wane over time and tends to worsen with sleep deprivation, caffeine intake, pregnancy, and alcohol. • Secondary causes = Renal dz, neuropathy, and Fe def. • In this pt, correcting the Fe def deficiency is the best choice for initial tx as this may entirely relieve the Sxs of RLS. • For pts with primary RLS (not related to another condition), the dopaminergic agents (Pramipexole and ropinirole) are the txs of choice.

21. Harrison’s Self-Assessment • A 38 yoAAF is referred to the clinic for evaluation of an abnl CXR (bilateral hilaradenopathy). She had been brought to the hospital after an MVC and had a CXR to evaluate for rib fx. She has since recovered from her accident with no further CP. She is o/w in good health and has had no SOB, cough, or wheezing. She has never had prior lung dz. She denies recent acute illness, fevers, chills, night sweats, or weight loss. She has a h/o HTN and takes lisinopril. She lives in West Virginia. She does not smoke. On exam, she appears well and in NAD. An O2 sat on RA is 97%. Her exam is o/w unremarkable. A CT of the chest is recommended and shows bilateral enlargement of hilarLNs and a R paratrachealLN measuring up to 1.5 cm. The lung parenchyma is NL. PFTs show a TLC of 4.8 L (96% predicted) and a DLCO of 13.4 (88% predicted). Spirometry is NL w/o obstruction. Bronchoscopy with transbronchial biopsies and transbronchial needle aspiration shows noncaseatinggranulomas. No fungal elements or AFB are seen, but cultures are pending. • What is the best approach to therapy for this patient? • A. Isoniazid, pyrazinamide, rifampin, and ethambutol • B. Itraconazole • C. Prednisone 20 mg daily • D. Prednisone 1 mg/kg daily • E. Reassurance and close follow-up

22. Sarcoidosis • Inflammatory d/o of unknown cause, characterized by noncaseatinggranulomas. • Typically occurs in young, otherwise-healthy adults. Up to 20% of cases can be found incidentally on CXR in aSx’c pts, as in this case. When present, typical Sxs are most commonly cough and dyspnea. However, sarcoidosis can affect any organ system. • After the respiratory Sxs, skin dz and ocular findings are the most common manifestations. • Lung involvement is seen in >90%, and staging is based on CXR findings. • Stage I - hilaradenopathy only. • Stage II - hilaradenopathy w/ pulmonary infiltrates. • Stage III - no evidence of hilaradenopathy, but interstitial pulmonary dz is present • Stage IV - pulmonary fibrosis. • Occasionally, the term stage 0 disease is used to refer to individuals with extrapulmonarysarcoidosis and no lung involvement. • Tx is largely based on Sxs, and no tx is needed in aSx’c pts. • In stage I disease, 50-90% will resolve spontaneously w/o tx. When tx is needed, prednisone is the initial tx of choice. Usually doses of 20–40 mg are effective, but with cardiac or neurologic involvement, higher doses (up to 1 mg/kg), are often needed. • For severe manifestations of sarcoidosis, addition of azathioprine, methotrexate, or cyclophosphamide may be required. • Joint and dermatologic manifestations often respond well to hydroxychloroquine.

23. Harrison’s Self-Assessment • A 22 yoM has CF. He currently is hospitalized about 3x per yr for infectious exacerbations. He is colonized with Pseudomonas and Staph aureus, but has never had Burkholderiacepacia. He remains active and is in college studying architecture. He requires 2 L O2 w/ exertion. His most recent PFTs show an FEV1 of 28% of predicted and an FEV1/FVC of 44%. ABG = 7.38 / 46 /62 on RA. • Which of these characteristics is an indication for referral for lung transplantation? • A. Colonization with Pseudomonas aeruginosa • B. FEV1 <30% predicted • C. FEV1/FVC ratio <50% • D. PaCO2 >40 mmHg • E. Use of oxygen with exertion

24. Pts w/ CF should be considered for lung transplant when • FEV1 is < 30% predicted or is rapidly falling. • PaO2 < 50 on RA • PaCO2 >50 • Pulmonary HTN • Inc’g hospitalization • Recurrent hemoptysis.

25. Harrision’s Self Assessment • Among the following pulmonary function test results, which is the most likely finding in chronic obstructive pulmonary disease? • A. Increased total lung capacity (TLC), decreased vital capacity (VC), decreased FEV1/FVC ratio • B. Decreased TLC, decreased VC, decreased residual volume (RV), increased FEV1/FVC ratio, normal maximum inspiratory pressure (MIP) • C. Decreased TLC, increased RV, normal FEV1/FVC ratio, decreased MIP • D. Normal TLC, normal RV, normal FEV1/FVC ratio, normal MIP

26. Dec’d FEV1/FVC ratio diagnoses obstructive lung dz. With extensive air trapping in OLD, TLC is often inc’d and RV may also be inc’d. VC is proportionally dec’d. • Alternatively, low lung volumes, specifically dec’d TLC, and occasionally dec’d RV diagnose restrictive lung dz.

27. Harrison’s Self Assessment • Among the following pulmonary function test results, which is the most likely finding in myasthenia gravis? • A. Increased total lung capacity (TLC), decreased vital capacity (VC), decreased FEV1/FVC ratio • B. Decreased TLC, decreased VC, decreased residual volume (RV), increased FEV1/FVC ratio, normal maximum inspiratory pressure (MIP) • C. Decreased TLC, increased RV, normal FEV1/FVC ratio, decreased MIP • D. Normal TLC, normal RV, normal FEV1/FVC ratio, normal MIP

28. MIP measures respiratory muscle strength and is dec’d in pts w/ NMSK dz. Thus, myasthenia gravis will produce low lung volumes and dec’d MIP

29. MKSAP Q111 • A 40 yoM is seen for the insidious onset of exertionaldyspnea and dry cough x a few mos. Sxs were preceded by an URI. He has a h/o childhood asthma and a 1 yr h/o arthralgias in his knee and finger joints. He has never smoked and has no fever, weight loss, or night sweats. He has 2 dogs and an indoor cat. He has traveled all over the US, Mexico, and Europe. He is a pediatrician and has been in his new office for 18 mos, where he has goldfish, a small turtle, a caged pair of cockatiels, rats, and hamsters in a contained area. On exam, he is well appearing, afebrile, and his BP is 136/70. The rest of his exam, including joint exam, is unremarkable. PFTs are as follows: • FVC = 60% predicted • FEV1 = 50% predicted • FEV1/FVC ratio = 0.82 • DLCO (single breath; corrected to Hgb) = 35% predicted • While breathing air, the pt’s O2 sat at rest is NL but dec’s to 91% while walking 500 feet on a flat surface and to 83% upon climbing 1 set of stairs. CXR shows NL cardiac size and diffuse pulmonary infiltrates in both lungs. • Which of the following procedures would provide a specific diagnosis in this patient? • A HRCT • B Bronchoscopy with BAL and transbronchial biopsy • C Serum precipitins to cockatiels • D Serologic testing for HIV • E Echocardiography

30. Interstitial Lung Dz • Characteristic clinical features = progressive dyspnea, diffuse radiographic pulmonary infiltrates, restrictive pulmonary physiology, and O2 desaturation with exertion. • HRCT scanning should be performed as part of the standard evaluation of ILD and some radiographic patterns can suggest a particular dx but HRCT is unlikely to provide a specific dx. • In the correct clinical setting, bronchoscopy with BAL can provide a specific dxin ILD. • Other answers • Serum precipitin testing would help establish the relationship b/w a specific exposure and an immunologic response; however the presence of precipitins is not dx’cof dzand exposed pts w/o lung dzcan have (+) precipitins. • Serologic testing for HIV would not provide a specific dxfor this pt's pulmonary problem. • Echo to assess his cardiac function is not indicated b/c he does not have Sxsof LHF or pulm HTN. (DOE and desats with exertion are nonspecific findings and c/w his ILD.)

31. MKSAP Q11 • A 75 yoF w/ a long h/o asthma is evaluated for inc’d nocturnal Sxs and frequent need to use an albuterol inhaler. Her tx regimen now consists of daily moderate-dose inhaled corticosteroids. On exam she has occasional wheezing; but the exam is o/w unremarkable. Office spirometry shows an FEV1 of 2.2 L (75% predicted). • Which of the following is the most appropriate adjustment to this patient's asthma therapy? • A Doubling the inhaled corticosteroid dose • B Adding theophylline • C Adding a leukotriene receptor antagonist • D Adding a long-acting β-agonist • E Adding anti-IgE antibody

32. Having asthma Sxs 2 or more days per week (or 2 or more nights per month) indicate that pt has persistent asthma. • Pts with persistent asthma should all be treated with daily inhaled corticosteroids. • When asthma is not adequately controlled on low- or moderate-dose inhaled corticosteroids, adding a long-acting β-agonist has been shown to be superior to doubling the corticosteroid dose in terms of improving asthma control and the patient's quality of life. • Theophyllineand leukotriene receptor antagonists are third-line drugs and should be considered in pts who are still Sx’cafter adding a long-acting β-agonist.

33. MKSAP Q 37 • A 38 yoF is evaluated for worsening control of mild-persistent asthma. Her dz had been under good control on tx with moderate-dose inhaled corticosteroids plus prn albuterol until 6 weeks ago when she had an acute respiratory tract infection. Since then she had significant worsening of her Sxs, with nightly cough and wheezing and use of albuterol rescue inhaler 6-8x per day. • Which of the following is the most appropriate tx for this pt? • A A 7-day course of a fluoroquinolone antibiotic • B Nebulized albuterol/ipratropium bromide at home • C A short course of oral corticosteroid therapy • D A leukotriene receptor antagonist

34. A short burst of oral corticosteroids (eg, Prednisone 0.5 mg/kg/day for 5-7 days) may help restore asthma control in previously well-controlled pts who have developed unstable dz d/t a respiratory tract infection • Other answers • ABXs are not recommended for acute respiratory infxs in asthma b/c most of these infxs are viral. • Adding an leukotriene receptor antagonist can be considered in patients who cannot (or will not) take oral corticosteroids; however, they are less potent anti-inflammatory agents than corticosteroids and may not be effective in patients with significant exacerbations. • Nebulized therapy at home should be reserved for those who cannot use an MDI appropriately. Even though they can be more effective in reversing bronchosconstriction than MDI bronchodilators, nebulized bronchodilator tx should not be used as a substitute for corticosteroid therapy in patients with asthma exacerbations.

35. Harrison’s Self-Assessment • 32 yoM is brought to the ED after developing sudden-onset SOB and CP while coughing. He reports a 3-month h/o inc’g DOE, nonproductive cough, and anorexia with 15 lb of weight loss. He has no PMH and takes no meds. He patient smokes 1-2 ppd, uses alcohol socially, and has no HIV risk factors. A CXR a R-sided 80% PTX, and there are nodular infiltrates in the L base that spare the costophrenic angle. After placement of a chest tube, a chest CT shows bilateral small nodular opacities in the lung bases and multiple small cystic spaces in the lung apex. • Which of the following interventions is most likely to improve the Sxs and radiograms? • A. Intravenous 1 antitrypsin • B. Isoniazid, rifampin, ethambutol, and pyrazinamide • C. Prednisone and cyclophosphamide • D. Smoking cessation • E. Trimethoprim-sulfamethoxazole

36. Pulmonary Langerhans Cell Histiocytosis (EosinophilicGranulomas) • Can be found incidentally on radiograms or may p/w respiratory and systemic complaints. • Spontaneous PTX is a common presentation and occurs in ~ 25% of these pts. • The radiographic combination of small reticular/nodular opacities in the bases (with sparing of the costophrenic angle) and apical cysts is characteristic and virtually diagnostic. • Cigarette smoking is virtually universal among these pts. • PFTs will show dec’d DLCO. Lung volumes may be NL or dec’d, depending on the severity. • ~33% of these pts improve w/ smoking cessation, but most develop progressive interstitial dz. • Immunosuppressive agents do not appear to influence the course of dz.