2. Case No. 1

3. • A 63-year-old woman has a 1-month history of dry cough,
night sweats, anorexia, and a 50-lb (23-kg) weight loss. She
is a nonsmoker. She has type 2 diabetes, dyslipidemia, and
hypertension but had stopped all medication 6 months
earlier after having a disagreement with her physician. She
did not abuse drugs or alcohol, and there was no history of
recent travel.
• Other than left basal crackles, results of a physical
examination were normal. Other than mild hyponatremia
(serum sodium level, 132 and hyperglycemia (glucose level,
490 mg/dL, results of laboratory studies were normal.
• Results of chest radiographs and representative CT images
are shown. Bronchoscopy demonstrated no endobronchial
lesions, cytologic studies were negative, and cultures grew
only normal respiratory flora.

6. At this point, you would initiate which of the
following interventions?
• A. Percutaneous drainage with a pigtail
catheter.
• B. Lung resection.
• C. Broad-spectrum antibiotics with anaerobic
coverage.
• D. Bronchoscopic abscess drainage.

7. • The chest radiographs and CT scan show a large
left lower lobe lung abscess with irregular thick
walls. While the appearance of the abscess could
be consistent with a malignancy, the clinical
presentation and negative bronchoscopy suggests
that this is a pyogenic lung abscess. The lack of
treatment for this patient’s diabetes likely played
a role. When an endobronchial obstruction has
been excluded, a prolonged course of broad-
spectrum antibiotics with anaerobic coverage is
the standard treatment.

8. • Only if this regimen fails, drainage or resection
options are considered. Percutaneous
drainage with a pigtail catheter is one option
but is not necessary or indicatedat this time.
Pneumonostomy or cavernostomy with direct
drainage (Monaldi procedure) is another
option but is only possible when the pleural
space is obliterated.Lung resection is usually
reserved for septic complications.These
surgical procedures achieved cure rates of
90% but with concomitant mortality rates of
• 11% to 28%.

9. • More recently, endoscopic drainage of a lung
abscess has been introduced. Under fluoroscopic
control, a guidewire is introduced into the cavity
through the working channel of a flexible
bronchoscope. Once the guidewire is confirmed
to be in place, the catheter and bronchoscope are
removed, and a 90-cm pigtail catheter of at least
7F is slipped over the wire into the cavity. This
form of drainage and percutaneous catheter
drainage has proven effective in appropriately
selected adult and pediatric patients but only if
antibiotic therapy has proved unsuccessful.

10. • Medical therapy is usually successful, and it has been estimated
that drainage is required in only 11% to 21% of patients.
• Given the CT appearance of the irregular, thick-walled cavity, careful
follow-up will be required to ensure complete resolution.
• Endoscopic drainage can be attempted in select patients who have
an airway connection to the abscess. It has an advantage over other
procedures in that it does not carry the risk of soiling the pleural
space and is less invasive and not associated with the loss of lung
parenchyma, as in a surgical resection.
• Oropharyngeal anaerobes and microaerophilic streptococci have
been the predominant pathogens involved in lung abscess. In some
studies, gram-negative bacteria have been the etiologic agent in as
many as 20% of patients. Therefore an appropriate initial treatment
regimen would include a third-generation cephalosporin with
clindamycin or metronidazole or possibly a -lactam/-lactamase
inhibitor alone.

11. At this point, you would initiate which of the
following interventions?
• A. Percutaneous drainage with a pigtail
catheter.
• B. Lung resection.
• C. Broad-spectrum antibiotics with anaerobic
coverage.
• D. Bronchoscopic abscess drainage.

12. Case No. 2

13. • A 68-year-old man is evaluated for a 1-month history of
hemoptysis. He had a history of stable schizophrenia,
• untreatedCOPD, and remote bladder cancer. He had
smoked 2 packages of cigarettes daily for the past 32
years but quit 4 months ago due to an increase in his
chronic cough and sputum production. He had a
chronic cough productive of at least 2 tbsp (30 mL) of
thick white-yellow mucus daily going back many years,
but for the previous 2 months, he had regular flecks of
pinkish material in his sputum. He was otherwise well.

14. • Physical examination revealed normal vital signs,
decreased breath sounds bilaterally, a prolonged
forced expiratory time, and high pitched, late
expiratory wheezes most marked over the upper
lobes. Results of preliminary blood work,
including a CBC, prothrombin time, and partial
thromboplastin time, were all normal. He had a
FEV1 of 1.1 L (55% of predicted) and FVC of 2.7 L
(95% of predicted), with an FEV1/FVC of 0.40 and
no response to inhaled bronchodilator. He had
hyperinfl ation, gas trapping, and a Dlco of 53% of
predicted

16. CT after 2 months

17. Which of the following is most true
about this condition?
• A. It has a high malignant potential.
• B. It is a consequence of granulomatous infl
ammation.
• C. Th oracotomy for removal is the treatment
of choice.
• D. It is more commonly found in the left lung.

18. • The initial CT scan reveals protrusion of a calcified lesion
into the bronchus intermedius. Bronchoscopy reveals a
whitish lesion protruding through the wall of the bronchus
intermedius. Finally, the repeat CT scan after 2 months
shows migration of the calcification into the right lower
lobe bronchus. It can no longer be seen in the bronchus
intermedius and now occludes the lateral and posterior
segmental bronchi to the right lower lobe. Together, these
findings are diagnostic of broncholithiasis. This most
commonly occurs as a result of infection with
Mycobacterium tuberculosis or Histoplasma capsulatum. A
repeat bronchoscopy confirms migration of the broncholith
to the right lower lobe.

20. • Broncholithiasis is defined as the presence of
calcified or ossified material within the lumen
of the bronchus. Most broncholiths originate
as calcifications of peribronchial lymph nodes
that subsequently erode into the airway. These
lymph nodes calcify after an inflammatory
process usually initiated by infection.
• TB is the most common cause of broncholiths.

21. • Broncholiths are much more common on the right side
perhaps related to the fact that lymphatic drainage of
the lung eventually reaches the right paratracheal and
azygous nodes. The most common symptoms include
cough and hemoptysis and, occasionally, actual stones
are coughed up (lithoptysis).
• Patients may develop recurrentpneumonias from
occluding stones, and the latter may migrate, as
occurred in this patient. Although calcifi ed tumors,
such as carcinoids, hamartomas, or osteosarcomas,
may mimic broncholiths, the latter do not have
increased malignant potential.

22. • Flexible bronchoscopy can be utilized to visualize the lesion but
does not have a therapeutic role. Rigid bronchoscopy can be used
to remove stones occasionally with the aid of neodymium-doped
yttrium aluminum garnet lasers. Older research cautioned against
bronchoscopic removal of broncholiths because of concern for
inducing massive hemoptysis/hemorrhage.
• Thoracotomy with segmental or lobar resection was the procedure
of choice. However, more recent literature has clearly shown that
the majority of broncholiths seen in the airway are mobile and can
be removed bronchoscopically, with thoracotomy reserved for the
minority of patients in whom the broncholiths are adherent to the
mucosa.
• Asymptomatic broncholiths do not require therapy, and patient
observation alone is recommended.

23. Which of the following is most true
about this condition?
• A. It has a high malignant potential.
• B. It is a consequence of granulomatous infl
ammation.
• C. Th oracotomy for removal is the treatment
of choice.
• D. It is more commonly found in the left lung.

24. Case No. 3

25. • A 25-year-old woman, primigravida, in her 25th week of pregnancy is seen
in your office for worsening asthma. She has had asthmasince childhood,
which has been managed with good control with fluticasone, 250 ug 2 puff
s bid, and salmeterol, 50 ug 1 puff bid, as well as prn salbutamol.
• She now reports several weeks of nightly nocturnal awakenings with
wheezing, cough, and chest tightness and is using her albuterol inhaler
more than 4 times a day. She denies symptoms of gastroesophageal reflux.
• On physical examination, she is in no respiratory distress with stable vital
signs. Her respiratory rate is 15/min. She is not using external muscles of
accessory ventilation. She is afebrile. Her lung examinationreveals diff use
wheezing, but with preserved air movement, and her abdominal
examinationis notable for a gravid appearance. Her peak flow rates are
approximately 60% of her personal best.

26. In addition to asthma education, the next best step in
her management would be which of the following?
• A. Begin oral corticosteroids.
• B. Begin tiotropium
• C. Double the dose of inhaled corticosteroids.
• D. Change fluticasone to budesonide.

28. • Asthma aff ects up to 8% of pregnant women and, if poorly
controlled, can result in increased fetal and maternal
morbidity, including increased risks of preterm labor,
preeclampsia, congenital anomalies, intrauterine growth
retardation, and placenta previa.
• In general, the course of asthma in pregnancy can be divided
into thirds, with one third of patients improving, one-third
worsening, and one-third remaining at baseline. The severity
of asthma in prior pregnancies and in the nonpregnant state
are good predictors of the course of asthma in pregnancy.
Asthmatends to be worse in the middle of pregnancy
(gestationalweeks 17 to 36) and better in the first and latter
third trimesters.

29. • Gastroesophageal reflux, sinusitis, and allergic
rhinitis, leading to worsening asthma, should be
carefully evaluated in a pregnant woman who has
asthma.
• The treatment of chronic asthma in pregnancy is
generally similar to that of the patient with asthma
who is not pregnant. The National Asthma Education
Program sponsored by the National Heart, Lung, and
Blood Institute developed guidelines for the
treatment of the pregnant person with asthma,
subsequently revised in 2004 and 2007.

30. • In general, patients with asthma whose asthma is well
controlled on an appropriate medical regimen prior to
pregnancy can remain on this regimen if control is acceptable
during pregnancy. This woman is a moderately controlled
(moderate persistent) asthmatic who now is falling into the
poorly controlled (severe persistent) category, with frequent
nocturnal awakenings and increased use of albuterol rescue
therapy. Systemiccorticosteroidswould be indicated at this
point in this patient as in a nonpregnant asthmatic.

31. • Systemiccorticosteroids are category C agents. There have
been several studies examining the effects of corticosteroidsin
pregnancy. Initial studies suggested that systemic
corticosteroidswere associated with prematurity, infants with
low birth weight, and an increase in congenitalmalformations,
including cleft palate. However, further review of these studies
postulated that poorly controlled asthma was the cause of the
majority of these abnormalities. Patients receiving systemic
corticosteroidsrequire careful evaluation for gestational
diabetes. In the patient with asthma who is corticosteroid
dependent, stressdose corticosteroids should be administered
during labor and delivery.

32. • Short-acting selective 2-agonists are indicated for well-
controlled (mild intermittent)asthma (albuterol has been the
drug most widely studied in pregnancy, and is the preferred
agent). Low-dose inhaled corticosteroidsare the preferred
agent for initiating treatment for partially controlled asthma
(mild persistent) in the pregnant patient.
• Budesonide (category B) and beclomethasone (category C) are
the best-studiedinhaled corticosteroids, and budesonide is
the preferred agent. Patients whose condition is being
successfullycontrolled with other inhaled corticosteroids can
continue using these agents.

33. • Alternative treatments include leukotriene receptor antagonists (not
zileuton) or low dose theophylline. For those whose asthmais less well
controlled (moderate persistent asthma), a medium dose inhaled steroid
or low-dose steroid with a long-acting β-agonist (salmeterol is preferred
due to adequacy of studies) is recommended. Poorly controlled (severe
persistent) asthma should be managed with high-dose inhaled
corticosteroids and a long-acting β 2-agonist, as with this patient, and an
oral corticosteroid should be added if needed.
• Short-acting selective 2-agonists are category C agents. Inhaled
corticosteroids are category B (budesonide) and C (all other inhaled
corticosteroids) agents. Salmeterol and formoterol are category C agents.
Th eophylline is a category C agent. The leukotriene antagonists,
montelukast and zafirlukast, although not well studied in pregnancy, are
category B agents.
• Zileuton (category C) is the least well studied and should be avoided.

34. • Th e other choices listed, although permitted during pregnancy, would not
be appropriate in a patient who now has worsening asthma. Th e patient
is already on a reasonable dose of inhaled corticosteroids and doubling
the dose would likely be of little benefit, although an increase in her
maintenance dose may be required once the exacerbation is better
controlled (choice C is incorrect). Although recent data maysuggesta
benefit of tiotropium in asthma, this is a category C agent with minimal
data for use in pregnancy.
• Changing to the category B inhaled corticosteroid would not be indicated
to improve poorly controlled asthma, and a change in inhaled steroid
because of pregnancy is not recommended in patients already on an
inhaled corticosteroid regimen.

35. In addition to asthma education, the next best step in
her management would be which of the following?
• A. Begin oral corticosteroids.
• B. Begin tiotropium
• C. Double the dose of inhaled corticosteroids.
• D. Change fluticasone to budesonide.

36. Case No. 4

37. • A 19-year-old man is referred to you for assessment of possible pulmonary
arteriovenousmalformations (PAVMs). The patient’sfather was recently diagnosed
with hereditary hemorrhagic telangiectasia (HHT) after having hemoptysis. The
father was subsequentlydocumented to have pulmonary arteriovenous
malformations, and the diagnosis was confi rmed by genetic testing. The son
denies any symptoms and is a lifelong nonsmoker. He gets occasionalnosebleeds,
but they are mild and stop spontaneously.He denies any shortness of breath or
activity limitation.He also denies any hematemesis, melena, or hematochezia.The
remainder of the history is noncontributory.
• Physicalexaminationrevealsmucosal and cutaneous telangiectasia.There is no
clubbing, cyanosis, pallor, or jaundice. Oxygen saturationon room air is 98%.
Results of a chest examination arenormal, and the rest of the physical
examination resultsare normal. The patient’shemoglobinlevel is 13.9 g/dL.
Results of liver enzymes and function tests are normal. The patient’schest
radiographis reported normal with no evidence of nodules, masses, or feeding
vessels.

38. Which would be the next most appropriate step
to assess for the presence of PAVMs?
• A. Measurement of the physiologic shunt
fraction.
• B. Pulmonary angiography.
• C. CT chest angiography.
• D. Contrast echocardiography.

39. • This patient has a high clinical suspicion for HHT (Osler-Rendu-Weber
Syndrome) with a confirmed positive family history, nosebleeds, mucosal
and cutaneous telangiectasia, and anemia. It would be important to
confirm the diagnosis of HHT with genetic testing. While the chest
radiograph is normal, the next mostimportant clinical step is to assess for
possible PAVMs. All patients with possible or confirmed HHT should be
actively screened for PAVMs.
• While careful clinical examinationshould routinely be undertaken, only
10% of affected patients have cyanosis, clubbing, and/or a pulmonary
bruit. The screening technique of choice is contrast echocardiography with
agitated saline, which is more sensitive than oxygen shunt testing.

40. • CT scanning should not routinely be used for screening because of the
associated radiation exposure (choice C is incorrect) unless expertise in
transthoracic contrast echocardiography is not readily available. Pulmonary
angiography has a lower resolution than current multidetector CT scanning
and should not be performed as a screening procedure.
• PAVMs are present in approximately 15% to 50% of people with HHT.
Acknowledging the risk and the frequency of serious complications, all
patients with HHT should be screened for pulmonary and cerebral
arteriovenous malformations. Complications include life-threatening
hemorrhage and neurologic complications. Stroke has been reported in
10% to 36% of patients, transient ischemic attack in 6% to 37%, cerebral
abscess in 8% to 19%, and massivehemoptysis or spontaneous
hemothorax in 4% to 20%. Th e hemorrhagic complications of PAVMs can
be life threatening, and the risk appears to be increased during pregnancy.

41. Which would be the next most appropriate step
to assess for the presence of PAVMs?
• A. Measurement of the physiologic shunt
fraction.
• B. Pulmonary angiography.
• C. CT chest angiography.
• D. Contrast echocardiography.

42. Case No. 5

43. Mutations of which of the following genes are
common in heritable pulmonary arterial
hypertension?
• A. Toll-like receptor (TLR).
• B. Bone morphogenetic protein receptor II
(BMPR2).
• C. Protease inhibitor (PI ).
• D. Epidermal growth factor receptor (EGFR).

44. • DNA specimens from families with pulmonary
arterial hypertension (PAH) are commonly found to
have mutations in the gene on chromosome 2q32
that encodes bone morphogenetic protein receptor II
(BMPR2 ); these genotypes occur in 80% of families
with heritable PAH (choice B is correct). Although this
is an autosomal dominant disorder, it is expressed in
only 20% of carriers, presumably related to
environmental factors or other genetic modifi ers.

45. • Genomics refers to the study of the genome that arose from technologies
that map and sequence genes and their relationships to cellular and
molecular biology. Proteomics, a related discipline, is the study of
proteins, how they are modified and expressed, and how they are involved
in metabolic pathways and other proteins.
• Well-known examples of genomic techniques that elucidated mechanisms
of disease include identifying mutations in the cystic fibrosis
transmembrane receptor (CTFR) in cystic fibrosis and the protease
inhibitor (PI ) gene in 1-antiprotease deficiency.
• Similarly, specific genetic variations in the Toll-like receptor pathway (TLR)
predict poor outcomes in sepsis (choice A is incorrect), and mutations of
the epidermal growth factor receptor (EGFR) gene identify a subset of
patients with adenocarcinoma of the lung who may respond clinically to
EGFR-inhibitor agents.

46. Mutations of which of the following genes are
common in heritable pulmonary arterial
hypertension?
• A. Toll-like receptor (TLR).
• B. Bone morphogenetic protein receptor II
(BMPR2).
• C. Protease inhibitor (PI ).
• D. Epidermal growth factor receptor (EGFR).

47. Case No. 6

48. • A 33-year-old man with longstanding cystic fibrosis was admitted to the
ICU 2 days ago with massive hemoptysis for which he was endotracheally
intubated and placed on ventilatory support. Chest CT scan revealed
bronchiectasis in both lungs but focal air fluid levels and extensive
bronchiectasis in the left lower lobe. Flexible bronchoscopy revealed
active hemorrhage from the left lower lobe without visible mucosal
lesions. He underwent bronchial arteriography with embolization of four
suspicious tortuous vessels.
• He has had no recurrence of hemoptysis for the past 48 h, during which
time he has remained sedated and paralyzed pharmacologically. Several
hours after cessation of neuromuscular blockade, he coughed vigorously
and now has recurrent massive hemoptysis with fresh blood filling the
endotracheal (ET) tube and ventilator tubing. Despite placing the patient
with his left side down and performing aggressivetracheal suctioning, his
oxygen saturation by pulse oximetry has rapidly fallen to 70% (with a good
waveform) while bag-ventilated with 100% oxygen, and his heart rate is
slowing.

50. Which of the following interventions
should be performed next?
• A. Rapid transfusionof packed RBCs.
• B. Flexible bronchoscopy with epinephrine
irrigation of bleeding site(s).
• C. Right mainstem bronchus intubation.
• D. Emergent arteriography and repeat
embolization.

51. • Massive hemoptysis is an immediately life-threatening condition, and this
patient has evidence of ongoing hemorrhage with flooding of the airway
resulting in profound hypoxemia with impending asphyxiation. Urgent
isolation of the bleeding lung units from functioning lung units that can
support gas exchange is the immediate priority. Blindly advancing the ET
tube into the right mainstem bronchus is the mostimmediately available
option because it is highly likely that the bleeding is again from the left
lower lobe, and the vastmajority of blind bronchial intubations are right
sided.

52. • Urgent flexible bronchoscopy through the advanced ET tube
should follow to confirm satisfactory placement, assist in
evacuation of blood, and exclude unexpectedright-sided
bleeding. Careful repositioning of the ET tube such that
aeration of the right upper lobe is possible should be
considered. Other methods of isolation of bleeding lung units
from functional lung include bronchoscopically-guided
placement of a catheter with an inflatable cuff , such as a
bronchial blocker or Fogarty balloon catheter, which can
occlude the bronchus to the active bleeding site. Double-
lumen ET tubes are effective at protecting the nonbleeding
lung but are difficult to insert properly and limit ability to
perform bronchoscopy or suction effectively.

53. • Hemorrhage-related life-threateninghypoxemia must be corrected as a priority
over other interventions.However, subsequently, bronchoscopymay be useful to
identify bleeding site(s), clear airway blood,perform balloon tamponade,and
provide topicalinterventionsto reduce active bleeding, such as application
• of epinephrine or thrombin, iced saline lavage,or electrocautery. Bronchial
arteriographyand embolization of suspect bronchialarteries is eff ective at
stopping active bleeding in most cases, but recurrence is common. Surgical
resection of lung with a localizedbleedingsite can be defi nitive for persistent or
recurrent bleeding. Surgery that is emergent, however, is associated with higher
rates of complicationsand mortality. Diff use underlyinglung disease, such as
cystic fi brosis, creates added concerns for subsequent recurrent bleeding from a
new site after lung resection. Additionalgeneralmeasures includecorrection of
abnormalitiesin coagulationand plateletnumber or function, as well as correction
of anemia from bleeding.

54. Which of the following interventions
should be performed next?
• A. Rapid transfusionof packed RBCs.
• B. Flexible bronchoscopy with epinephrine
irrigation of bleeding site(s).
• C. Right mainstem bronchus intubation.
• D. Emergent arteriography and repeat
embolization.

55. THANK YOU