MCQ Pneu
MCQ Pneu
MCQ Pneu
The answer is b.
The clinical picture suggests hypertrophic
osteoarthropathy. This process, the pathogenesis
of which is unknown, is characterized by clubbing
of digits, periosteal new bone formation, and
arthritis. Hypertrophic osteoarthropathy is
associated with intrathoracic malignancy,
suppurative lung disease, and congenital heart
problems. Treatment is directed at the underlying
disease process. While x-rays may suggest
osteomyelitis, the process is usually bilateral and
easily distinguishable from osteomyelitis. The first
step in evaluation of this patient is to obtain a
chest xray looking for lung infection and
carcinoma. The process is periarticular, not
articular; so septic arthritis, treated with
parenteral antibiotics, would not be a
consideration. Although there is warmth over the
wrists, the clubbing and periosteal changes would
not be seen in rheumatoid arthritis, so wrist
The answer is c.
Milky pleural fluid associated with high pleural fluid
triglyceride level (above 110) indicates chylothorax,
usually caused by disruption or compression of the
thoracic duct. Hence, most chylous effusions are leftsided. Trauma is the commonest cause, but in this
patient, lymphoma should be strongly considered. The
lymphocytes in the pleural fluid may be monoclonal in
origin. Flow cytometry of these cells or biopsy of one
of the accessible peripheral lymph nodes will reveal
the diagnosis. Tuberculosis can cause a chylous
effusion but would typically be associated with
parenchymal lung disease. Generalized
lymphadenopathy would be unusual unless the TB
were associated with AIDS. Lung cancer would usually
be accompanied by a parenchymal mass and would
rarely cause chylothorax. Congestive heart failure
usually causes bilateral effusions; if unilateral, the
effusion in CHF is almost always right sided. In
addition, unless the patient has had vigorous diuresis,
CHF causes a transudate (not an exudate). A
parapneumonic effusion is exudative, but is seen in
The answer is c
Because clinical signs of neurologic
deterioration and a petechial rash have
occurred in the setting of fracture and
hypoxia, fat embolism is the most likely
diagnosis. This process occurs when neutral
fat is introduced into the venous circulation
after bone trauma or fracture. The latent
period is 12 to 36 hours. A pulmonary
embolus usually has a longer latent period. In
addition, pulmonary embolus would not
cause the petechial rash. Confusion out of
proportion to the degree of hypoxemia is also
seen with fat emboli. Unilateral pulmonary
edema can be seen with aspiration and after
rapid expansion of a pneumothorax, but not
The answer is c.
This patient presents with severe COPD and
hypoxemia. Chronic CO2 retention has blunted his
hypercarbic drive to breathe; he is dependent on mild
hypoxia to stimulate respiration. An inappropriately
high oxygen delivery has decreased even that drive,
with resulting acute respiratory acidosis and CO2
narcosis. However, stopping the oxygen will result in
severe hypoxemia. Of the choices listed, the initiation
of mechanical ventilation is the only acceptable
choice. If the patients mental status were better,
noninvasive ventilation (BiPAP) might be considered.
Medroxyprogesterone has only a mild stimulatory
effect on the respiratory center, and is not appropriate
therapy in this case. Antibiotics and inhaled
bronchodilators are appropriate treatments for COPD
exacerbation but would not manage this patients
acute hyper-carbic respiratory failure. The patient has
declared a deteriorating course. Continuing to monitor
his status on the general medicine ward would
probably be fatal. This patient has respiratory (not
metabolic) acidosis. Bicarbonate plays a minimal role
The answer is b.
Classifying a pleural effusion as either a transudate or
an exudate is useful in identifying the underlying
disorder. Pleural fluid is exudative if it has any one of
the following three properties: a ratio of concentration
of total protein in pleural fluid to serum greater than
0.5, an absolute LDH greater than 2/3 the upper
normal in serum, or a ratio of LDH concentration in
pleural fluid to serum greater than 0.6 (the Light
criteria). Causes of exudative effusions include
malignancy, pulmonary embolism, pneumonia,
tuberculosis, abdominal disease, collagen vascular
diseases, sarcoidosis, uremia, Dressler syndrome, and
chylothorax. Exudative effusions may also be drug
induced. If none of the aforementioned properties are
met, the effusion is a transudate. Differential diagnosis
for a transudative effusion includes congestive heart
failure, nephrotic syndrome, cirrhosis, Meigs syndrome
(benign ovarian neoplasm with effusion), and
hydronephrosis. Exudative effusions are the result of
an inflammatory process causing proteins to leak
across the capillary membrane. Transudative effusions
The answer is c.
Patients with cystic fibrosis are now surviving into
adulthood. The median survival is approximately age
41. Most cases are diagnosed in childhood; however,
because of variable penetration of the genetic defect,
approximately 7% are not found until the patient is an
adult. Most male patients (> 95%) are azoospermic.
Chronic pulmonary infections occur, and
bronchiectasis frequently develops. Diabetes mellitus
and gastrointestinal problems indicate pancreatic
insufficiency. This patient should have sweat chloride
measurement; if abnormal (sweat Cl above 70 mEq/L),
cystic fibrosis transmembrane conductance regulator
(CFTR) mutation analysis should be ordered. COPD or
emphysema at this age would be unusual unless the
patient were deficient in alpha-1 antitrypsin.
Immunoglobulin deficiencies can cause recurrent
sinopulmonary infections but would not cause
malabsorption or infertility. Whipple disease causes
malabsorption but not the pulmonary manifestations
or infertility; it would be vanishingly rare in a young
patient. Asthma would not cause the abdominal
The answer is e.
Occupational lung disease is an important branch of pulmonology,
and new inhaled workplace toxins are being described every year. A
detailed occupational history and knowledge of potential culprits
are, therefore, critical in patients with unexplained lung disease.
This patients exposure history is suggestive of asbestos related
disease; bilateral pleural thickening (often calcified, a finding
especially evident on CT scan) indicates prior asbestos exposure.
Occasionally, the pleural involvement is associated with a pleural
effusion (often with an elevated red cell count) called benign
asbestos pleural effusion (BAPE). Benign distinguishes this
syndrome from malignant effusions due to lung cancer or
mesothelioma, both of which occur with increased frequency in
asbestosis. Progressive debility from an interstitial lung disease
(which worsens even after asbestos exposure has ceased) may
occur in asbestosis, but this patients physical examination and
chest x-ray do not suggest interstitial disease.
Medications, especially nitrofurantoin and cancer chemotherapeutic
agents, can cause interstitial lung disease, but amlodipine has not
been reported to do so. Tuberculosis occurs with increased
frequency in silicosis, not in asbestosis. Although TB can cause a
bloody pleural effusion, this patient does not have the systemic
symptoms that usually accompany TB. Hypersensitivity pneumonitis
is an important cause of occupational lung disease, but it is caused
by exposure to organic materials such as thermophilic
actinomycetes (farmers lung). In addition, hypersensitivity
pneumonitis usually causes acute symptoms (including fever) at
The answer is b.
Because of the development of effective oral antibiotics (respiratory
fluoroquinolones, extended spectrum macrolides), most patients with
community-acquired pneumonia (CAP) can be managed as an outpatient as
long as compliance and close followup are assured. The CURB-65 score is
the most strongly validated instrument for determining if inpatient
admission (either observation or full admission) is indicated. Factors
predicting increased severity of infection include confusion, urea above
19mg/dL, respiratory rate above 30, BP below 90 systolic (or 60 diastolic),
and age above 65. If more than one of these factors is present,
hospitalization should be considered.
This patients presentation (lobar pneumonia, pleuritic pain, purulent
sputum) suggests pneumococcal pneumonia. Pneumococci are the
commonest organisms isolated from patients with CAP. Fortunately, S
pneumoniae is sensitive to oral antibiotics such as
clarithromycin/azithromycin and the respiratory quinolones. A Gram stain
suggestive of pneumococci would therefore only confirm the clinical
diagnosis. Exposure to influenza is an important historical finding. However,
without a prodrome of influenzalike illness (upper respiratory symptoms,
myalgias, prostrating weakness), this is still garden variety CAP. In the
setting of an influenza-like illness, H influenzae (easily treated with standard
antibiotics) and S aureus pneumonia (more problematic to treat) must be
considered. Acute lobar pneumonia, even in an HIV-positive patient, is due
to the pneumococcus and can be treated as an outpatient. Pneumocystis
jirovecii pneumonia is usually insidious in onset, causes diffuse parenchymal
infiltrates, and does not cause pleurisy or pleural effusion. Physical
examination signs of consolidation confirm the CXR finding of a lobar
pneumonia (as opposed to a patchy bronchopneumonia) and would simply
affirm the importance of coverage for classic bacterial pathogens (ie,
pneumococci, H flu). Atypical pneumonias (still often pneumococcal, but
The answer is b.
The clinical situation strongly suggests pulmonary embolism. In
greater than 80% of cases, pulmonary emboli arise from thrombosis
in the deep venous circulation (DVT) of the lower extremities, but a
normal lower extremity Doppler does not exclude the diagnosis.
DVTs often begin in the calf, where they rarely if ever cause clinically
significant pulmonary embolic disease. However, thromboses that
begin below the knee frequently grow, or propagate, above the
knee; clots that dislodge from above the knee cause clinically
significant pulmonary emboli. Untreated pulmonary embolism is
associated with a 30% mortality rate. Interestingly, only about 50%
of patients with DVT of the lower extremities have clinical findings of
swelling, warmth, erythema, pain, or palpable cord. When a clot
does dislodge from the deep venous system and travels into the
pulmonary vasculature, the most common clinical findings are
tachypnea and tachycardia; chest pain is less likely and usually
indicates pulmonary infarction. The ABG is usually abnormal, and a
high percentage of patients exhibit low PCO2 with respiratory
alkalosis, and a widening of the alveolar-arterial oxygen gradient.
The ECG usually shows sinus tachycardia, but atrial fibrillation,
pseudoinfarction in the inferior leads, and acute right heart strain
are also seen. Initial treatment for suspected pulmonary embolic
disease includes prompt hospitalization and institution of
intravenous heparin or therapeutic dose subcutaneous lowmolecular-weight heparin. It is particularly important to make an
early diagnosis of pulmonary embolus, as intervention can decrease
the mortality rate from 30% down to 5%. A normal D-dimer level
The answer is a.
This patient likely has primary pulmonary hypertension.
Echocardiogram is a reliable noninvasive test to confirm the
clinical suspicion. Once pulmonary hypertension is confirmed,
secondary causes (pulmonary or congenital heart disease)
should be ruled out. These are unlikely in this patient without
clinical or radiographic evidence of chronic pulmonary
disease. Once pulmonary hypertension is confirmed by
echocardiography and secondary causes ruled out, patients
often undergo right heart catheterization with measurement
of pulmonary vascular resistance in response to various
pulmonary vasodilators. Treatment choices have expanded in
recent years; bosentan, sildenafil, and in severe cases, infused
prostacyclin are effective treatments. In refractory cases,
heart-lung transplantation (with its considerable risks) may be
necessary. Spirometry is useful in defining obstructive or
restrictive lung disease. Spirometry will be normal in
pulmonary hypertension. Exercise stress testing in this patient
will show a nonspecific decline in exercise tolerance; it is
diagnostically useful when ischemic heart disease is a
consideration. Measurement of alpha-1 antitrypsin would be
indicated if this young woman had obstructive lung disease,
but none of her clinical features point in this direction. If COPD
were causing her symptoms, O2 desaturation or radiographic
evidence of hyperexpansion would be expected.
The answer is c.
Effective prophylaxis against DVT in the
high-risk setting (eg, after major
orthopedic surgery of the hip or knee)
requires pharmacologic treatment with
unfractionated heparin, low-molecularweight heparin, fondaparinux, or
therapeutic doses of warfarin.
These treatments, when given at approved
dosages and time intervals, decrease the
risk of radiographic DVT by over 50%;
dosage guidelines should be carefully
followed. Aspirin alone is not effective in
prevention of pulmonary embolus. Early
ambulation, sequential compression
The answer is a.
This patient with multiple episodes of desaturation has
obstructive sleep apnea (OSA). In OSA, upper airway muscle
tone decreases as the patient achieves deep stages (stages 3
and 4) of sleep; the soft palate falls against the base of the
tongue, leading to obstruction of air flow and snoring.
Microawakening occurs, leading to improvement in muscle
tone but at the cost of shallow unrefreshing sleep. This leads
to daytime somnolence (due to sleep deprivation),
hypertension (due to hyperadrenergic state), and even cor
pulmonale and chronic hypercarbia (due to hypoxia). At
present fewer than five apneic episodes per hour are
considered normal. The severity of sleep apnea is graded
using the apnea/hypopnea index. Mild sleep apnea is 5 to 15
events per hour; moderate sleep
apnea is 16 to 30; severe apnea is greater than 30 events per
hour Continuous positive airway pressure is the recommended
therapy. Weight loss is often helpful and should be
recommended as well. However, weight loss alone will take
significant time and may not be sufficient.
Uvulopalatopharyngoplasty, when applied to unselected
patients, is effective in less than 50%.
A trial of CPAP is indicated before surgical therapy.
Tracheostomy is a treatment of last resort in severe and
The answer is b.
Asthma is an inflammatory process with
reversible airflow obstruction. This patients
presentation suggests exercise-induced
asthma. Asthma is an incompletely
understood disease that involves the lower
airways and results in bronchoconstriction
and excess production of mucus. This, in turn,
leads to increased airway resistance and
occasionally respiratory failure and death. In
any obstructive lung disease such as chronic
obstructive pulmonary disease, hyperinflation
may be present on chest x-ray and FEV1 may
be decreased. Only in asthma is the airway
obstruction fully reversible. Hypoxia would be
unusual in exercise-induced asthma and
would suggest an alternative diagnosis.
Reduced forced vital capacity (FVC)
The answer is d.
This patients chronic cough, hyperinflated lungs,
abnormal pulmonary function tests, and smoking
history are all consistent with chronic bronchitis. A
smoking cessation program can decrease the rate
of lung deterioration and is successful in as many
as 40% of patients, particularly when the
physician gives a strong antismoking message and
uses both counseling and nicotine replacement.
Continuous low-flow oxygen becomes beneficial
when resting arterial oxygen saturation falls below
88%. Inhaled beta agonists or anticholinergics
such as ipratropium or tiotropium are the
cornerstones of symptomatic therapy but do not
prevent progression of airways obstruction if the
patient continues to smoke. Antibiotics are
indicated only for acute exacerbations of chronic
lung disease, which present with fever, change in
sputum color, and increasing shortness of breath.
Oral corticosteroids are helpful in acute
The answer is c.
Hyponatremia in association with a lung mass usually
indicates small cell lung cancer (SCLC) with inappropriate ADH
production by the tumor. About 10% of lung cancers present
with a paraneoplastic syndrome. Tumors producing ADH or
ACTH are overwhelmingly SCLCs, which arise from hormonally
active neuroendocrine cells. SCLC is a rapidly growing
neoplasm; early mediastinal involvement, as in this case, is
common. Tumor staging for SCLC differs from nonsmall cell
cancers. SCLCs are simply classified as limited (confined to
one hemithorax) or extensive. Limited tumors are usually
managed with combination radiation and chemotherapy, with
approximately 20% cure rate. Extensive tumors are treated
with palliative chemotherapy alone; durable remissions are
rare. Surgery is not curative in SCLC.
Bronchial carcinoids are usually benign. Although they can
produce ACTH, mediastinal involvement and hyponatremia
would not be expected. Adenocarcinoma of the lung, although
common, rarely causes a paraneoplastic syndrome. Localized
benign lung infections (especially lung abscess) can cause
SIADH, but would not account for this patients mediastinal
adenopathy. Lung abscess usually causes fever and fetid
sputum. Pulmonary aspergilloma (a fungus ball growing in an
old cavitary lesion) can cause hemoptysis but not this
The answer is e.
This patient has interstitial lung disease (ILD) due to her systemic sclerosis.
Over 75% of patients will have CT evidence of ILD. Now that scleroderma
renal crisis can be managed with ACE inhibitors, ILD is the most common
disease related cause of death in scleroderma. The two most important
studies for the diagnosis of ILD are spirometry with measurement of
diffusion capacity of carbon monoxide (DLCO) and high-resolution CT scan.
The latter will show reticular interstitial thickening and subpleural
microblebs. Advanced cases will show thickened fibrotic bands with
parenchymal destruction known as honeycombing. Treatment of connective
tissue disease associated ILD is unsatisfactory, but cyclophosphamide will
slow progression in some patients. In addition to systemic sclerosis, ILD is
an important potential complication of polymyositis/dermatomyositis,
rheumatoid arthritis, and occasionally systemic lupus. An arterial blood gas
study would probably show alveolar hyperventilation (ie, low PCO2) with a
widened alveolar-arterial oxygen gradient but would not give specific
information as to cause and prognosis. In most cases, finger oxygen
saturation measurements provide sufficient information. Pulmonary
hypertension can be detected with 2D echocardiogram according to the
degree of tricuspid regurgitation. Pulmonary hypertension can be an
important complication of connective tissue disease (especially limited
scleroderma but sometimes diffuse scleroderma as well). You would expect,
however, a loud P2 and evidence of central pulmonary artery enlargement
on CXR. In addition, Velcro rales and increased interstitial markings are not
seen in uncomplicated pulmonary hypertension. Autoantibodies are usually
found in systemic sclerosis, but the diagnosis is already established. The
titer of anti-Scl antibodies does not correlate with disease activity; so once
the diagnosis is established, serial measurement of autoantibodies is not
necessary. Aspiration due to esophageal dysmotility can complicate
scleroderma, but usually causes intermittent exacerbations associated with
The answer is c.
Clues to this diagnosis are recurrent urinary tract infections
and the use of suppressive therapy to control these infections.
Nitrofurantoin is commonly used for this purpose.
Nitrofurantoin can cause an acute hypersensitivity
pneumonitis. This condition can progress to a chronic alveolitis
with pulmonary fibrosis. The presenting symptoms are fever,
chills, cough, and bronchospasm. In addition, the patient may
experience arthralgias, myalgias, and an erythematous rash.
The chest x-ray will show interstitial or alveolar infiltrates. CBC
often shows leukocytosis with a high percentage of
eosinophils. The treatment is to discontinue the nitrofurantoin,
and to begin corticosteroids. Sepsis secondary to a urinary
tract infection and healthcare-related pneumonia might be
considered. However, these would not present with a diffuse
erythroderma or eosinophilia. Acute bacterial infections cause
a neutrophilic leukocytosis; eosinophils are usually
undetectable owing to the stress effect of catecholamines and
cortisol. COPD rarely presents in a 30-year-old. Lymphocytic
interstitial pneumonia is a rare disease and would cause
interstitial rather than alveolar infiltrates. Lung biopsy to
establish the diagnosis of an interstitial lung disease would be
considered only after the potentially offending drug had been
discontinued.
The answer is a.
Although a difficult diagnosis to make, primary
pulmonary hypertension is the most likely
diagnosis in this young woman who has used
appetite suppressants. Primary pulmonary
hypertension in the United States has been
associated with fenfluramines. The predominant
symptom is dyspnea, which is usually not
apparent until the disease has advanced. When
physical findings, chest x-ray, or echocardiography
suggest pulmonary hypertension, recurrent
pulmonary emboli must be ruled out. In this case,
a normal perfusion lung scan makes pulmonary
angiography unnecessary. Right-to-left cardiac
shunts cause hypoxia (oxygen desaturation) that
characteristically does not improve with oxygen
supplementation. Restrictive lung disease should
be ruled out with pulmonary function testing but is
unlikely with a normal chest x-ray. An
echocardiogram will show right ventricular
The answer is e.
With the history of daytime sleepiness and snoring
at night, the patient requires evaluation for
obstructive sleep apnea syndrome. Frequent
awakenings are actually more suggestive of
central sleep apnea. Polysomnography is required
to assess which type of sleep apnea syndrome is
present. EEG variables are recorded to identify
various stages of sleep. Arterial oxygen saturation
is monitored by finger or ear oximetry. Heart rate
is monitored. The respiratory pattern is monitored
to detect apnea and whether it is central or
obstructive. Outpatient sleep monitoring with
oxygen saturation studies alone might identify
multiple episodes of desaturation, but negative
results would not rule out a sleep apnea
syndrome. Overnight oximetry alone can be used
in some patients when the index of suspicion for
The answer is b.
For suspected pulmonary embolism, CT with intravenous
contrast has surpassed the ventilation-perfusion scan as the
diagnostic method of choice. New multislice scanners can
detect peripheral as well as central clots. Lung scanning may
be useful in selected circumstances. PE is very unlikely in
patients with normal or near-normal scans, and is highly likely
in patients with high-probability scans. In patients with a high
clinical index of suspicion for pulmonary embolus but lowprobability scan, the diagnosis becomes more difficult.
Catheter-based contrast pulmonary angiography (the gold
standard) may occasionally be necessary but is not the first
step. About two-thirds of patients with pulmonary embolus
have evidence of deep venous disease on venous ultrasound.
Therefore, pulmonary embolus cannot be excluded by a
normal study. The quantitative Ddimer enzyme-linked
immunosorbent assay is positive in 90% of patients with
pulmonary embolus. It has been used to rule out PE in
patients with a low-probability scan. A contrast CT study is
needed, however, in patients with intermediate or high pretest
probability of pulmonary embolism. High-resolution CT scan of
the chest is useful in the diagnosis of interstitial disease but
does not adequately assess pulmonary vasculature; IV
contrast is necessary to diagnose PE.
The answer is c.
Postoperative atelectasis or volume loss is a very
common complication of surgery. General
anesthesia and surgical manipulation lead to
atelectasis by causing diaphragmatic
immobilization. Atelectasis is usually basilar. On
physical examination, shift of the trachea to the
affected side suggests volume loss. On chest x-ray
in this patient, loss of the left hemidiaphragm,
increased density, and shift of the hilum
downward would all suggest left lower lobe
collapse. Atelectasis needs to be distinguished
from acute consolidation of pneumonia, in which
case fever, chills, and purulent sputum are more
pronounced and consolidation is present without
volume loss.
Volume loss would not be a feature of a spaceoccupying mass, bronchospasm, or
The answer is c.
With symptoms of headache and dizziness in
a fireman, the diagnosis of carbon monoxide
poisoning must be addressed quickly. A
venous or arterial measure of
carboxyhemoglobin must first be obtained, if
possible, before oxygen therapy is begun.
The use of supplementary oxygen prior to
obtaining the test may be a confounding
factor in interpreting blood levels. Oxygen or
even hyperbaric oxygen is given after blood
for carboxyhemoglobin is drawn.
Methemoglobinemia causes cyanosis, which
is not present in this patient. EKG is unlikely
to be abnormal in this young healthy patient
without chest pain. Central nervous system
imaging would not be indicated, and there
are no diagnostic patterns that are specific to
The answer is b.
Theophylline has been used as a bronchodilator for a
number of years. It has been less commonly used in
recent years owing to its narrow therapeutic window.
The drug is metabolized in the liver. A drug or process
that interferes with the activity the cytochrome P450
system will slow the metabolism of theophylline and
may lead to the accumulation of toxic levels in the
blood. The metabolism of theophylline is slowed by
age, infection, CHF (resulting from decreased hepatic
blood flow), and a number of drugs. Commonly used
drugs that impair the metabolism of theophylline
include cimetidine, erythromycin, ciprofloxacin,
allopurinol, and zafirlukast. This patient has probably
been using over-the-counter cimetidine to treat her
reflux symptoms. Stopping theophylline until the drug
level has returned will relieve her palpitations and
tremor. In the absence of dyspnea, wheezing, or
clinical signs of CHF, chest film and spirometry would
not be helpful. Using a benzodiazepine to treat her
tremor would leave a potentially serious theophylline
toxicity undetected.
The answer is a.
This patient has stage IIB non-small cell lung cancer
(NSCLC) and should be considered for surgical
resection with curative intent. As a general rule,
patients with stages I and II lung cancer are surgical
candidates unless other medical contraindications or
severe COPD are present. Adjuvant chemotherapy is
sometimes added, but surgery is the curative modality
with the best track record. Patients with stage I lung
cancer have tumors localized to the lung. Stage II
cancers are associated with ipsilateral peribronchial or
hilar lymph node involvement. Mediastinal lymph node
involvement, pleural effusion, or distant metastases
generally preclude curative surgery. These patients,
however, may respond to radiation and/or
chemotherapy.
Although some patients have achieved long-term
remission after radiation therapy, it is less effective
than surgical resection. Combination chemotherapy
can prolong life expectancy in selected patients but is
not considered curative. Endobronchial radiation
The answer is d.
This woman has COPD (chronic symptoms, obstructive defect on
spirometry) at age younger than 45. Early-onset symptoms, even in
a smoker, coupled with a positive family history, should raise the
possibility of alpha-1 antitrypsin (AAT) deficiency, and a serum AAT
level should be ordered. If it is low, a phenotype assay will confirm
the abnormal gene product. AAT deficiency tends to cause more
prominent alveolar destruction in the lower lung zones, as opposed
to usual smokers emphysema, which has an upper lobe
predominance. Diagnosing AAT deficiency would be important for
her family members. In addition, infusion of pooled human AAT,
although quite expensive, can raise AAT levels and probably slows
progression of the disease.
Cystic fibrosis, which is diagnosed by the sweat chloride level, can
present with lung disease in adulthood. However, this womans lack
of cough and sputum production, as well as her normal fertility,
makes this a less likely diagnosis than AAT deficiency. Diffusing
capacity will be low in any cause of emphysema, and CT scanning
will confirm bullous changes, but neither is recommended in the
routine management of COPD. High-resolution CT scanning is used
in the diagnosis of interstitial, not obstructive, lung disease. This
woman has a history of exposure to organic compounds known to
cause hypersensitivity pneumonitis, but her lack of symptoms
during or soon after exposure, as well as the absence of patchy
infiltrates on CXR, makes this diagnosis less likely. Many agricultural
workers have immunoprecipitins to thermophilic actinomycetes. In
the absence of convincing history, these results are
The answer is a.
Chronic cough is a common problem encountered in the clinic.
Although patients are often worried about serious disease
such as lung cancer, emphysema, or tuberculosis, these dire
diagnoses are rare in the absence of a compatible history and
chest x-ray. The commonest causes are (1) acid reflux (with
inflammation of the larynx and trachea); (2) postnasal drip
syndrome; (3) cough-variant asthma; and (4) drug-induced
cough due to angiotensin-converting enzyme inhibitors
(ACEIs). Extensive testing such as CT scanning, bronchoscopy,
or esophageal pH monitoring is not recommended. The
practitioner should make the best diagnosis on the basis of
initial data, and then institute a therapeutic trial,
understanding that a response often takes weeks or months.
This patients nocturnal symptoms and occasional
postprandial reflux (patients often do not have severe
symptoms of GERD) would direct you toward a trial of PPIs. If
she had allergic symptoms or cobblestoning of the posterior
pharynx, a trial of nasal steroids/decongestants would be
reasonable. Childhood asthma, intermittent wheezing, or
exacerbation of symptoms with exercise or cold exposure
would direct you toward a therapeutic trial of bronchodilators.
Spirometry is usually normal unless the patient is having
symptoms at the time of examination; methacholine challenge
can be employed in selected patients. Amlodipine does not
The answer is b.
This young man is suffering from high-altitude
pulmonary edema (HAPE), a life-threatening condition.
It is the commonest nontraumatic cause of death in
high-altitude climbers. Susceptible persons have
increased pulmonary vasoconstriction in response to
hypoxia; this damages capillary endothelium and leads
to exudation of fluid into the alveoli. Patients with
HAPE are at significant risk of recurrence. The
cornerstone of treatment is oxygen administration
and/or descent to lower altitude, where the partial
pressure of oxygen is higher. HAPE is a form of
noncardiogenic pulmonary edema; left ventricular
function is normal. Although nifedipine decreases
pulmonary vascular tone and is a useful adjunct, relief
of hypoxia is a much more important aspect of
treatment. Rapid ascent, sleeping at high altitude, and
individual susceptibility are important risk factors;
interestingly, youth and physical conditioning are not
protective (perhaps because young people have
vigorous pulmonary vascular reactivity).
Acetazolamide (a carbonic anhydrase inhibitor) is
The answer is c.
Oxygen treatment (as close to 24 hours a day as
possible) is the one active treatment modality that has
been shown to decrease mortality in COPD.
Interestingly, it decreases the incidence of sudden
death. This effect is presumably due to the beneficial
effect of oxygen on cor pulmonale and right heart
strain. It is important to emphasize to the patient that
they should use the oxygen continuously, not just at
times of increased dyspnea. Several treatments
(inhaled corticosteroids, long-acting bronchodilators)
are symptomatically useful and may slow progression
of functional loss but have not been shown to prolong
life. Pulmonary rehabilitation can increase functional
status but does not improve parameters such as FEV1
or mortality. The number of exacerbations is an
important determinant of functional decline in COPD,
but preventing them is difficult. Prompt antibiotic
treatment of purulent exacerbations decreases the
rate of hospitalization but has not been proven to
affect mortality. Methods to slow progression of COPD
The answer is e.
The Global initiative for chronic Obstructive Lung Disease
(GOLD) guidelines recommend the use of spirometric
values to standardize the diagnosis and staging of COPD.
The diagnosis emphasizes a compatible history and
evidence of fixed or incompletely reversible airway
obstruction as demonstrated by a ratio of FEV1 to FVC
less than 70%. Restrictive lung disease causes a
proportional decline in both FEV1 and FVC; so the ratio of
the two will remain normal.
The stage of COPD is then determined by the decrease in
FEV1 (see table).