Transcript Document

Pulmonary Board Review
Questions from MKSAP and Harrison’s SelfAssessment
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
 In pts w/ potentially operable non-small-cell lung CA, PFTs are
indicated to assess pulmonary reserve.
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?
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A Bronchoscopy with lung biopsy
B Positron-emission tomographic scan
C Allergy skin testing
D Methacholine challenge test
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.
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?
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A Bronchoscopy with endobronchial biopsy
B Methacholine challenge test
C Inhalation challenge with increasing concentrations of chlorine
D Spirometry before and after work shift
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 hx and confirmed by a (+) methacholine challenge.
 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.
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?
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A CMV pneumonia
B Acute organ rejection
C Bronchiectasis
D PCP pneumonia
E Bronchiolitis obliterans
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.
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
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.
GOLD Staging of COPD
Stage
Characteristics
0: At risk
Normal spirometry
Chronic symptoms (cough, sputum production)
I: Mild
FEV1/FVC <70%
FEV1 ≥80% predicted
With or without chronic symptoms (cough, sputum production)
II: Moderate
FEV1/FVC <70%
50% ≤FEV1 <80% predicted
With or without chronic symptoms (cough, sputum production)
III: Severe
FEV1/FVC <70%
30% ≤FEV1 <50% predicted
With or without chronic symptoms (cough, sputum production)
IV: Very Severe
FEV1/FVC <70%
FEV1 <30% predicted or FEV1 <50% predicted plus chronic respiratory failure
* Classification based on post-bronchodilator FEV1
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
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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.
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.
Harrison’s Self-Assessment
 A 45 yoM is evaluated in clinic for asthma. Sxs began 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. Sxs are worst on weekdays but he cannot pinpoint
specific triggers. His medications are Albuterol MDI,
Fluticasone MDI, 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?
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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 Aspergillus fumigatus
 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.
Harrison’s Self-Assessment
 A 71 yoM p/w cough and sputum production. He occasionally coughs up a
small amount of blood. His Sxs have 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
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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.
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 ciliary dysfnx.
 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.
Harrison’s Self-Assessment
 A 45 yoF p/w abnl sensations 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 Sxs as "ants crawling in her veins." The Sxs are
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 Sxs disappear
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 neuro or
renal dz. She is currently perimenopausal and 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
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.
Harrison’s Self-Assessment
 A 38 yoAAF is referred to the clinic for evaluation of an abnl CXR (bilateral hilar
adenopathy). 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 hilar LNs and a R
paratracheal LN 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 noncaseating granulomas. 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
Sarcoidosis
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Inflammatory d/o of unknown cause, characterized by noncaseating granulomas.
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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.
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After the respiratory Sxs, skin dz and ocular findings are the most common manifestations.
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Lung involvement is seen in >90%, and staging is based on CXR findings.
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Stage I - hilar adenopathy only.
Stage II - hilar adenopathy w/ pulmonary infiltrates.
Stage III - no evidence of hilar adenopathy, but interstitial pulmonary dz is present
Stage IV - pulmonary fibrosis.
Occasionally, the term stage 0 disease is used to refer to individuals with extrapulmonary
sarcoidosis 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.
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 Burkholderia cepacia. 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?
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A. Colonization with Pseudomonas aeruginosa
B. FEV1 <30% predicted
C. FEV1/FVC ratio <50%
D. PaCO2 >40 mmHg
E. Use of oxygen with exertion
 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.
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
 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.
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
 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
MKSAP Q111
 A 40 yoM is seen for the insidious onset of exertional dyspnea 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:
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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?
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A HRCT
B Bronchoscopy with BAL and transbronchial biopsy
C Serum precipitins to cockatiels
D Serologic testing for HIV
E Echocardiography
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 dx in
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’c of dz
and exposed pts w/o lung dz can have (+) precipitins.
 Serologic testing for HIV would not provide a specific dx for this pt's pulmonary
problem.
 Echo to assess his cardiac function is not indicated b/c he does not have Sxs of LHF or
pulm HTN. (DOE and desats with exertion are nonspecific findings and c/w his ILD.)
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
 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.
 Theophylline and leukotriene receptor antagonists are third-line drugs and
should be considered in pts who are still Sx’c after adding a long-acting βagonist.
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 moderatedose 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
 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.
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 Rsided 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?
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A. Intravenous 1 antitrypsin
B. Isoniazid, rifampin, ethambutol, and pyrazinamide
C. Prednisone and cyclophosphamide
D. Smoking cessation
E. Trimethoprim-sulfamethoxazole
Pulmonary Langerhans Cell Histiocytosis (Eosinophilic
Granulomas)
 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.