Interactive case presentations //

Case Studies
Gamal Rabie Agmy, MD, FCCP
Professor of chest Diseases, Assiut university

• A 27-year-old man is referred to you for evaluation of an abnormal
chest radiograph. About 5 months ago, he consulted a doctor
because of excessive thirst. Evaluation resulted in the diagnosis of
diabetes insipidus, which responded favorably to desmopressin
administered nasally. Recently, he started to notice shortness of
breath when climbing stairs, and a chest radiograph was obtained.
• Patient history reveals significant tobacco smoking, up to two packs
daily, for at least 14 years. The patient noticed the shortness of
breath for at least 2 years, and recently, he noted a point of
tenderness over the chest wall, lateral to the posterior axillary line
on the left.
• Oxygen saturation is 94% while breathing room air, and the rest of
his vital signs were normal. Auscultation reveals only rare crackles
without prolongation of the expiratory phase. There is a point of
tenderness over the left sixth and seventh ribs in the posterior
axillary line, and a chest CT scan is obtained.

The most likely diagnosis is:
• A. Metastatic tumor of unknown primary site.
• B. Sarcoidosis.
• C. Langerhans cell histiocytosis.
• D. Idiopathicpulmonary fibrosis.

• This patient exhibits mostof the recognized features of pulmonary
Langerhans cell histiocytosis (LCH), previously known as eosinophilic
granuloma and histiocytosis X: pulmonary changes involving the middle
and upper parts of the lungs with irregularly shaped cystic and nodular
lesions of varying size; involvement of a flat bone (the rib); and diabetes
insipidus in a young person who smokes (choice C is correct). The only
frequently en countered complication that is not present in this patient is
a history of spontaneous pneumothorax. The radiographic findings,
especially when a CT scan shows cystic and nodular lesions.
• Cystic, nodular, and fibrotic lesions seen on CT scans are the most
frequent findings, followed by the presence of bone lesions in up to 20%
of patients, and diabetes insipidus in up to 15% of patients. Spontaneous
pneumothorax occurs in 15% to 25% of patients, although not in this
patient. Other manifestations contributing to morbidity and mortality
include pulmonary hypertension and the development of a pulmonary
neoplasm. The most important treatment for pulmonary LCH is cessation
of smoking.

• Glucocorticoids and immunosuppressiveagents have not been proven to
be effective. Smoking cessation alone will not alleviate pain in the
symptomaticrib lesions. Radiation therapy has been useful in controlling
progression and reducing pain. Similarly, smoking cessation alone will not
control diabetes insipidus; therefore, replacement therapy should
continue.
• The bone lesion could be due to a metastaticcancer, but the chest
radiograph does not show distinct nodules and is not consistent with
metastaticcancer. Patients with sarcoidosis may have diabetes insipidus
and may have extensive fibrosis late in the disease, but bone lesions are
rare (choice B is incorrect). While the fibrotic lesions maybe similar to
those found in patients with idiopathic pulmonary fibrosis (IPF), bone
lesions are not found in IPF and diabetes insipidus is not associated with
IPF.

Lung Cysts
By
Gamal Rabie Agmy , MD , FCCP
Professor of Chest Diseases ,Assiut University

Lung Cysts
Differential Diagnosis
Pulmonary fibrosis (Honeycombing)
Lymphangliomyomatosis
Langerhans cell histiocytosis
Lymphocytic Interstitial Pneumonia (LIP)
Birt-Hogg-Dubé syndrome (BHDS)

Rough Reticular Fine Reticular
Traction
Bronchiectasis
and
Interface
sign
Honey
combing
UIP UIP or NSIP

Usual Interstitial Pneumonia
UIP
HRCT Findings
Reticular opacities, thickened intra- and
interlobular septa
Irregular interfaces
Honey combing and parenchymal distorsion
Ground glass opacities (never prominent)
Basal and subpleural predominance

Basal and subpleural
distribution
UIP

The Many ‘HRCT Faces’ of NSIP
Honeycombing not
a
prominent feature
!!!!

Lymphangioleiomyomatosis
(LAM)
HRCT Morphology
Thin-walled cysts (2mm - 5cm)
Uniform in size / rarely confluent
Homogeneous distribution
Chylous pleural effusion
Lymphadenopathy
in young women

Lymphangioleiomyomatosis
(LAM)

Tuberous Sclerosis (young man)

Langerhans Cell Histiocytosis
HRCT Findings
Small peribronchiolar nodules (1-5mm)
Thin-walled cysts (< 1cm),
Bizarre and confluent
Ground glass opacities
Late signs: irreversible / parenchymal fibrosis
Honey comb lung, septal thickening,
bronchiectasis

1 year later
Peribronchiolar Nodules Cavitating nodules and cysts

Langerhans Cell Histiozytosis
Key Features
Upper lobe predominance
Combination of cysts and noduli
Characteristic stages
Increased Lung volume
Sparing of costophrenic angle
S
M
O
K
I
N
G

Differential Diagnosis
Only small nodules
Sarcoidosis, Silicosis
Only cysts
idiopathic Fibrosis
LAM
Destructive emphysema

.......after cessation of smoki

Benign lymphoproliferative
disorder
Diffuse interstitial infiltration of
mononuclear cells
Not limited to the air ways as
in follicular Bronchiolitis
LIP = Lymphocytic Interstitial
Pneumonia

LIP = Lymphocytic Interstitial
Pneumonia
Rarely idiopathic
In association with:
Sjögren’s syndrome
Immune deficiency syndromes, AIDS
Primary biliary cirrhosis
Multicentric Castlemean’s disease

Sjoegren disease
Dry eye and dry mouth
Fibrosis, bronchitis and bronchiolitis
LIP
Overlap
Sarcoid, DM/PM, MXCT
SLE, RA (pleural effusion)
Up to 40 x increased risk for lymphoma (mediastinal
adenopathy) and
2 x times increased risk for neoplasma

Young woman Dry mouth Smoker
LAM LIP Histiocytosis

History of recurrent pneumothorax, lung cysts, and skin
lesions (fibrofolliculomas) with normal lung function is
consistent with the diagnosis of Birt-Hogg-Dubé
syndrome
Birt-Hogg-Dubé syndrome

• BHDS is an autosomal dominantly inherited
genodermatosis that predisposes a person to the
development of cutaneous hamartomas (fi
brofolliculomas), kidney neoplasms, lung cysts, and
spontaneous pneumothorax.
• The BHD locus has been mapped to the short arm
of chromosome 17(17p11.2). BHD is composed of
14 exons, and more than 40 unique mutations in
BHD have been reported.
• Most BHD germline mutations are frameshift or
nonsense mutations that are predicted to truncate
the BHD protein, folliculin.

Mosaic pattern and its
differential

Where is the pathology ???????
in the areas with increased density
meaning there is ground glass
in the areas with decreased density
meaning there is air trapping

Pathology in black areas
Airtrapping: Airway
Disease
Bronchiolitis obliterans (constrictive bronchiolitis)
idiopathic, connective tissue diseases, drug reaction,
after transplantation, after infection
Hypersensitivity pneumonitis
granulomatous inflammation of bronchiolar wall
Sarcoidosis
granulomatous inflammation of bronchiolar wall
Asthma / Bronchiectasis / Airway diseases

Airway Disease
what you see……
In inspiration
sharply demarcated areas of seemingly increased
density (normal) and decreased density
demarcation by interlobular septa
In expiration
‘black’ areas remain in volume and density
‘white’ areas decrease in volume and increase in
density
INCREASE IN CONTRAST
DIFFERENCES
AIRTRAPPING

Hypersensitivity pneumonitis
Extr. Allerg. Alveolitis (EAA) HRCT
Morphology
chronic: fibrosis
Intra- / interlobular septal thickening
Irregular interfaces
Traction bronchiectasis
acute - subacute
acinar (centrilobular) unsharp densities
ground glass (patchy - diffuse)

Pathology in white Areas
Alveolitis / Pneumonitis
Ground glass
desquamative intertitial pneumoinia (DIP)
nonspecific interstitial pneumonia (NSIP)
organizing pneumonia
In expiration
both areas (white and black) decrease in
volume and increase in density
DECREASE IN CONTRAST
DIFFERENCES

Mosaic Perfusion
Chronic pulmonary embolism
LOOK FOR
Pulmonary hypertension
idiopathic, cardiac disease, pulmonary
disease

CTEPH =
Chronic thrombembolic
pulmonary hypertension

• An 81-year-old man comes to an outside hospital complaining
of abdominal pain. The patient has a CT scan of the abdomen
showing bilateral hydronephrosis, small bowel obstruction,
and a new bladder mass. The patient undergoes surgical
removal of the bladder mass, reported to be transitional cell
carcinoma, and is discharged several days later. The patient
now comes to your ED complaining of no urine output for 3
days and abdominal distension without any other symptoms.
•
• The patient’s past medical history is significant for congestive
heart failure. On physical examination, the patient appears
comfortable. The examination is notable for normal vital
signs, decreased breath sounds in the lower one half of his
right hemithorax, nontender abdomen with hypoactive bowel
sounds, and pitting edema to the knees bilaterally.

• Lab studies show: elevated brain-type natriureticpeptide (BNP)of
1,450 pg/mL (1,450ng/L), BUN of 44 mg/dL (15.7 mmol/L), creatinine
of 9.0 mg/dL (796 mol/L), WBC count of 7,000/L(7.0 × 109/L),
hematocrit of 33% (0.33), and platelet count of 277 × 103/L (277×
109/L). ABGs show a pH of 7.41, Pco2 of 39 mm Hg, and Pao2 of 72
mm Hg. His echocardiogram shows a left ventricular EF of 10% and
global hypokinesis.
• A CT scan of the chest, abdomen, and pelvis is obtained. The reported
CT findings include a right-sided pleural effusion, prominence of right
retroperitoneal fluid collection, and bilateral prominence of the renal
pelvises.
• Thoracentesis of the right pleural effusion reveals clear yellow fluid
with a protein level of 1.1 g/dL (11.0 g/L) (serum: 5.2 g/dL, 52.0 g/L),
lactatedehydrogenase (LDH) level of 75 U/L (1.25 kat/L) (serum: 224
U/L, 3.7 kat/L), glucose level of 93 mg/dL (5.2 mmol/L), pH of 7.20, and
WBC count of 46/mm3 (0.046 × 109/L).

Which of the following is the best option to treat
this patient’s pleural effusion?
• A. Diuresis.
• B. Broad-spectrum antibiotics.
• C. Bilateral nephrostomy tubes.
• D. Therapeutic thoracentesis.

• This patienthas a right-sided, low pH transudativepleuraleffusion compatible
with a urinothorax,further supported by evidence of bilateral obstructive
uropathyseen on CT scan, and it is best treated by relief of the obstruction (choice
C is correct). Urinothoraxis noted as the only cause of low-pH transudativepleural
effusion, as in this patient,althoughthe pleuralfluid pH may be normal.
• Urinothorax is the presence of urine within the pleuralspace most often due to
bilateralobstructiveuropathyor trauma. Thoracentesisprovides significantclues,
includingthe effusion being transudativewith a low pH, as in this patient.The
pleuralfluid smelled like urine, as was noted in this patientbut not reported in the
case summary. Glucose values may be normal or reduced and the protein level is
usually 1 g/dL (10 g/L). The LDH level may be high, misleading the clinicianfrom
the diagnosis. The pleuralfluid to serum creatinine ratiois 1.0 (median value of
4.0), with this finding being sensitive but not specific. This patient’spleuralfluid
creatininelevel was 22 mg/dL (1,945 mol/L), with a pleural fluid to serum
creatinineratio of 2.4.

• The two most likely pathways for development are bilateral
obstruction of the urinary tract caused by bilateral ureteral
or distal obstruction, as in this patient, and trauma to the
urinary tract (often iatrogenic and acute) that may be
unilateral.
• Both cause extravasation of urine that moves
retroperitoneally and ultimately into the pleural space.
• Although this patient likely has congestive heart failure as
evidenced by an elevated BNP level and an ejection fraction
of 10%, diuresis will not specifically treat the urinothorax.
• The patient has no evidence of infection, including being
afebrile and having a normal WBC count; therefore,
antibiotics are not warranted . The patient’s effusion is
asymptomatic, and the primary treatment is relief of his
obstructive uropathy; therefore, therapeutic thoracentesis
is not indicated.

• A 47-year-old woman has had severe asthma for
many years. She currently is receiving
salmeterol/fluticasone, 500 g bid; albuterol, 1 to 2
inhalations prn; and additional beclomethasone.
• Despite this therapy, she has had two asthma
exacerbations in the past year, requiring oral
corticosteroids, and one hospitalization.
• She has documented allergy to house dust mites
and cats, but environmental therapy in the past has
not been particularly helpful.
• Her FEV1 was 64% of predicted. Her serum IgE level
was 300 IU/mL (normal, 30 IU/mL). You
contemplate initiating therapy with omalizumab.

Which of the following statements
would be correct?
• A. An IgE level of 68 IU/mL would be
predictive of an excellent response to therapy
(normal, 30 IU/mL).
• B. ED visits will be reduced.
• C. The dosage administered is dependent only
on body weight.
• D. Clinical response can be judged within 2
weeks.

• This patient has poorly controlled asthma despite
the use of combination therapy even with
additional inhaled corticosteroids. She has an
elevated IgE level and, thus, would be an ideal
candidate for a trial of omalizumab.
• In a randomized prospective trial of add-on
omalizumab in 419 patients with inadequately
controlled, severe, persistent allergic asthma
despite optimized care, patients treated with
omalizumab had a reduction in the rate of ED
visits by 44% in addition to many other benefits.

• Pretreatment clinical characteristics poorly defi
ne the patient who is likely to respond to
omalizumab. The baseline serum IgE level has
only a broad predictive value. Patients with levels
below 75 IU/mL tend to have relative poor
responses when examining ED visits, asthma
quality-of-life questionnaires, and FEV1 as
outcomes, although there may be a reasonably
good response in severe exacerbation rates and
physician global assessment.

• Clinical benefi ts with omalizumab are seen when serum-
free IgE levels are 20.8 IU/mL (50 ng/mL) after the
initiation of therapy. Th e ability of omalizumab to achieve
these levels is dependent on dose, the patient’s weight,
and baseline serum total IgE level.
• A dosing table has been designed to account for body
weight and serum IgE level. A plateau of improvement in
asthma symptoms and morning peak expiratory flow has
been seen between 12 to 16 weeks after treatment onset.
About 40% of patients will demonstrate a response within 4
weeks of initiating treatment, whereas more than 60% will
have a response by 16 weeks (choice D is incorrect). Of the
patients who responded by 16 weeks, less than two-thirds
will have shown a response by 4 weeks, suggesting that a
trial of therapy should continue for at least 4 months.

• A 40-year-old woman comes for evaluation of progressive dyspnea on
exertion. Her symptomsstartedinsidiously 1 year ago and have
progressed to the point that she can climb less then one fl ight of stairs or
walk one city block at a quick pace. She denies nocturnal symptoms. She
has a nonproductive cough but does not have any other symptoms.
• Her past history is remarkable only for hypothyroidism for which she takes
thyroxine. She is a nonsmoker with no recent travel, traditional HIV risk
factors, or infectious contacts.
• Her physical examinationis remarkable only for slight tachypnea and an
arterial oxygen saturation of 89% at rest. Saturation decreases to a low of
79% during a 6-min walk. A chest radiograph was reported as abnormal,
which led to performing a CT scan (Fig 52-A). Bronchoscopy and
bronchoalveolar lavage(BAL) fl uid analysis were unhelpful, so a
• video-assisted thoracoscopic biopsy was performed.

Which of the following statements
about this condition is true?
• A. A trial of prednisone therapy is warranted
initially.
• B. Granulocyte-macrophage colony-stimulating
factor (GM-CSF) subcutaneous therapy works
best in patients with a predisposing condition.
• C. Inhaled GM-CSF is the primary treatment
modality.
• D. The patient should be offered whole lung
lavage.

• The CT scan (see Fig 52-A) reveals a diff use,
geographic, crazy paving pattern suggestive, but not
diagnostic, of pulmonary alveolar proteinosis (PAP).
The biopsy reveals diff use intra-alveolar filling by
granular proteinaceous material in which there are
sharply delineated round empty spaces, cholesterol
clefts, and scattered foamy macrophages and intra-
alveolar granular proteinaceous material, with adjacent
area of intraalveolar foamy macrophages and
interstitial chronic inflammation. Together, these
findings are characteristic of PAP.
• The treatment of choice is whole lung lavage .
Prednisone has no beneficial effect in PAP and would
be contraindicated given the increased prevalence of
disseminated, opportunistic infections in this disease.

• Anti-GM-CSF antibodies have been found in the serum
and BAL fl uid in patients with idiopathic PAP, leading
to the use of subcutaneous GM-CSF therapy of the
disease. However, patients with PAP secondary to an
underlying condition (eg, hematologic malignancies,
immunoglobulin defi ciency, HIV infection) do not have
these antibodies and, therefore,there is no role for
GM-CSF therapy.
• Although there are reports of successful use of inhaled
GM-CSF in PAP, response rates are generally lower than
with whole lung lavage, and this would be considered
salvage therapy should lavage not work.

• A 25-year-old African American woman is referred for increasing
shortness of breath and cough. She notes dyspnea on exertion
(one-block walk), increasing over the past 6 months. She does not
have chest pain, hemoptysis, fever, or weight loss. She recalls a
“lung collapse” last year that required placement of a “tube” to
correct the problem.
• She also recalls, more recently, some “fluid around her lung” that
was white in appearance. She had since been lost to follow-up. She
has a 10 pack-year history of tobacco use and has been trying to
quit. Physical examination is notable for stable vital signs and
bilateral crackles on lung examination. There is no clubbing. Her
chest radiograph reveals increased reticular opacities. Her CT scan is
shown in Figures 58-A and 58-B. Results of laboratory studies are
• unremarkable.

The most likely underlying histopathologic
finding in the lung is which of the following?
• A. Smooth muscle proliferation along the
lymphatics, interstitium, and bronchovasculature.
• B. Alternating areas of normal lung, interstitial
inflammation, fibroblastic foci, and honeycomb
changes.
• C. Centrally scarred stellate nodules with a
polymorphic infl ammatory infiltrate.
• D. Noncaseating granulomas in the interstitium
and around the vessels and bronchi.

• This patient has findings consistent with the diagnosis of
lymphangioleiomyomatosis (LAM). LAM is a rare disease
• (prevalence 1 to 2 per million) affecting premenopausal
women with a mean age of 35 years. The disorder is
• characterized by proliferation of atypical smooth muscle in
the bronchovasculature, lymphatics, and interstitium of the
• lung (choice A is correct), as well as in the abdomen and
pelvis. Typically, these smooth-muscle cells stain positive
with the monoclonal antibody HMB-45 (a melanoma-
related tumor marker).

• Clinically, patients have symptoms of dyspnea, cough, chest pain,
and hemoptysis. The physical examination may reveal decreased
breath sounds, crackles, and sometimes ascites and/or abdominal
masses. Spontaneous pneumothoraces and/or chylothoraces may
also be found. Radiographically, LAM is characterized by cyst
formation and chronic reticular opacities. Hyperinflation develops
as the disease progresses. Pleural effusions and pneumothoraces
may also be present. CT scanning can reveal diff use, homogenous
cystic disease, with cysts ranging from a few millimeters to 1 cm in
size (6-cm cysts have been reported); characteristically, nodularity is
absent. Pulmonary function testing most commonly shows mixed
obstructive and restrictive physiology with an elevated total lung
capacity and residual volume and a reduced diff using capacity, but
up to one-third of patients can have normal pulmonary function.

• Complications of LAM include recurrent pneumothoraces in 50% of
patients, chylous effusions in approximately one-third of patients,
chylous ascites, and renal angiomyolipomas in up to 50% of
patients. These latter tumors can develop in the kidneys or in other
solid organs (uterus, ovaries, liver, and spleen) and are composed of
blood vessels, smooth muscle, and fat.
• Treatment for LAM has included hormonal manipulation with the
use of antiestrogens, progesterones, and/or oophorectomy with
variable results. Lung transplantation has been performed for LAM,
although disease recurrence has been documented. Recently,
sirolimus, acting via suppression of mammalian target of rapamycin
(mTOR) signaling, has been studied with a primary endpoint of
decreasing angiomyolipoma volume.
• An interesting secondary finding was an improvement in spirometry
of 5% to 10% in FEV1 and FVC, which was only partially sustained
after discontinuation of sirolimus. The prognosis with LAM is
variable, but median survival is often reported to be 8 to 10 years.
The cause of death is usually respiratory failure.

• Pathologic evidence of sarcoidosis is characterized by
noncaseating granulomas in the interstitium and around
vessels and bronchi. The chest CT reveals hilar and
mediastinal lymphadenopathy, beading or irregular
thickening of the bronchovascular bundles, nodules along
bronchi, vessels, and subpleural regions, and occasionally,
ground glass opacifi cation. Pneumothoraces and pleural
effusions are uncommon (choice D is incorrect).
• Pathology of Langerhans cell histiocytosis (choice C) is
characterized by the presence of Langerhans cells,
eosinophils, lymphocytes, neutrophils, and alveolar
macrophages predominantly in the interstitium and around
bronchovascular structures, forming centrally scarred
stellate nodules

• A 65 year-old retired auto mechanic had a
chest radiographduring a preoperative
assessment for a cholecystectomy.

Which of the following statements is correct
concerning the likely underlying diagnosis?
• A. The patient is at an increased risk of
developing interstitial lung disease.
• B. Biopsy of one of the lesions would likely
reveal several ferruginous bodies.
• C. The underlying abnormality most
commonly involves the visceral pleura.
• D. The lesions are at high risk of
transformationto malignant mesothelioma.

• The patient’s chest radiograph reveals the classic changes
associated with benign pleural plaques associated with asbestos
exposure. Given that the patient has had enough exposure to
asbestos, likely through brake work in his job as an auto mechanic,
he is also at risk for developing asbestosis, which is lung fibrosis
caused by asbestos dusts.
• Both pleural and interstitial lung disease are related to the
magnitude and duration of exposure to asbestos. With the
exception of benign asbestos effusion, which can occur within a few
years of initial exposure, most
• manifestations tend to occur after 20 to 30 years of exposure.
Pleural and interstitial diseases often coexist, with pleural changes
reported in up to 80% of patients with asbestosis. In fact, classic
pleural plaques allow for a relatively firm clinical diagnosis of
asbestosis when interstitial changes are present.

• Pleural plaques are the most common
manifestation of asbestos exposure. They are
found in a higher proportionof male than
female patients, and the incidence of pleural
plaques increases with increasing age. Pleural
plaques are discrete areas of fibrosis that
typically arise from the parietal pleura. On
rare occasion,they may arise from the visceral
pleura, but this is not the typical pattern.

• The classic distribution of the plaques on chest
radiograph is the posterolateral chest wall
between the seventh and 10th ribs, the lateral
chest wall between the sixth and ninth ribs, and
the dome of the diaphragm (virtually
pathognomonic for asbestos plaques). The apices
and costophrenic angles are typically spared. CT
scan findings will support this distribution and
may also reveal paravertebral plaques not seen
well on chest radiographs.

• Calcification, as seen in this patient, is reported in 10% to 15% of
cases and becomes more prevalent with long-standing plaques. On
gross inspection, plaques are white, shaggy growths originating
from the inner surface of the rib. Histologic examination reveals a
relatively acellular “basket-weave” pattern of collagen bundles.
Asbestos fibers are often seen, but asbestos bodies are typically
absent. An asbestos body is an asbestos fiber that has been coated
in an iron-rich material; therefore, they also are called ferruginous
bodies. Pleural plaques do not typically cause symptoms, and most
data suggest no adverse impact on pulmonary function. The current
preponderance of evidence suggests that they do not undergo
malignant transformation.
• Other pleural diseases associated with asbestos exposure include
benign pleural effusions, diffuse pleural thickening (which, in
contrast, predominantly involves the visceral pleura and can cause
significant ventilatory impairment), rounded atelectasis, and
malignant mesothelioma. Patients exposed to asbestos are also at
increased risk of developing bronchogenic carcinoma, particularly if
they are current or past smokers, which has a synergistic effect on
risk.

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