Download Chronic Eosinophilic Pneumonia and Allergic Bronchopulmonary Aspergillosis: Two eosinophilic lung diseases in one patient

Andres Miranda
Gillian Lieberman, MD
August, 2011
Chronic Eosinophilic Pneumonia and Allergic Bronchopulmonary
Aspergillosis: Two eosinophilic lung diseases in one patient.
Andrés F. Miranda
Universidad de Antioquia, Faculty of Medicine
Gillian Lieberman, MD.
Andres Miranda
Gillian Lieberman, MD
August, 2011
Agenda
•
Index case presentation.
•
Eosinophilic Lung Diseases.
o Chronic eosinophilic pneumonia.
• Presentation.
• Diagnostic studies.
• Imaging.
• Differential diagnosis.
o Churg-Strauss Syndrome.
o Bronchiolitis obliterans organizing pneumonia.
o Simple pulmonary eosinophilia (Loeffler syndrome).
•
Patient additional work-up.
•
Allergic bronchopulmonary aspergillosis.
•
Case analysis.
•
Conclusions.
•
References.
•
Acknowledgements.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Index patient presentation.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: History of present illness
• 40 year old female reports for consultation with
cough, weight loss, abnormal CXR, and sputum
growing AFB.
• She c/o worsening chronic cough; clear sputum,
Some DOE. She has sustained an 15 pounds weight
loss over the past year. Denies hemoptysis.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Review of systems
•
Prominent malaise; fatigue; more than 30 pounds weight loss
over past several years.
•
Denies SOB at rest; no stridor, intermittent wheeze.
•
Denies fevers, chills, or sweats.
•
Denies chest pain, chest pressure, or palpitations.
•
No abdominal pain; no NVD
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Past medical history
•
Abnormal CXR in India 2005: biapical scarring.
•
Not clear if previous history of asthma.
•
BCG vaccination.
•
No medications.
•
No Known drug allergies.
•
No recent epidemiologic exposures.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Physical examination
• General: Cachectic, no acute distress.
• Vital signs: HR: 120, Sat: 91-93, T: 98.8.
• Lungs: diffuse rhonchi; diffuse coarse rales.
• Cardiac, abdominal, neurologic, extremities and
skin were within normal limits.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Initial Work‐up
• WBC was 9.1 with 57segs, 21 lymphs, and 13.8 eos.
• ESR: 30.
• AFB culture negative one month before
consultation.
• Recently Negative PPD
• Of course !!!! CXR.
• HIV, Hept B, Hept C and liver function tests were
within normal.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Pleural thickening, consoloditation and
reticulonodular pattern on Initial PA CXR
• Bilateral zones of pleural thickening and consolidation, predominant in the periphery. Furthermore a diffuse reticulonodular
pattern is present. PACS, BIDMC
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: parenchimal
densities Initial CXR lateral
•
Diffuse parenchimal
densities more
accentuated in
upper lobes.
PACS, BIDMC
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Andres Miranda
Gillian Lieberman, MD
August, 2011
• What is the differential diagnosis of a patient with
history of general and chronic respiratory symptoms,
marked peripheral eosininophilia and diffuse
findings on CXR ?
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Eosinophilic Lung Diseases
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Andres Miranda
Gillian Lieberman, MD
August, 2011
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Chronic Eosinophilic
Pneumonia
(CEP)
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CEP: Presentation
• Insidious onset and progressive.
• General manifestations:
o Asthenia, weight loss, nocturnal sweat or fever could be frequent.
• Pulmonary signs:
o Dyspnea, cough, wheezing could be present.
• Physical examination: Hypoxemia, tachycardia,
rales and wheezes.
• Twice more frequent in women.
• 1/3 previous history of asthma.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CEP: Diagnostic studies
Laboratory findings
• Market peripheral eosinophilia.
• ESR and CRP usually elevated.
• IgE leves elevated in 50% of the cases.
• Bronchoalveolar lavage (BAL): Allways high
percentage of eosinophils.
• Restrictive pattern or obstructive on lung function
tests could be found.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #1: Upper lobes consolidation on CXR
Areas of air space consolidation. Which are predominant in the upper lobes.
Yeon J, et al. Eosinophilic Lung Disease: A clinical, radiolic and pathologic overview, RadioGraphics. 2007
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #2: migratory infiltrates on CXR
Migratory pulmonary infiltrates, zones of consolidation
distributed peripherally.
Mendes L, et al. Pulmonary eosinophilia, The Jornal Brasileiro de Pneumologia. 2009
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #3: “Photographic
negative of pulmonary edema” CXR
UpToDate, 2011
The peripheral distribution of abnormalities results in the
typical finding of “Photographic negative of pulmonary
edema.”
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #4: subpleural consolidation and pleural effusion on Chest CT
C – axial chest CT. Lung window
Other common findings are subpleural areas of consolidation in both lungs. Right pleural effusion is also seen, but is observed in less than 10%.
Yookyung Kim, et al. The spectrum of eosinophilic lung disease: radiologic findings. Journal of computer assisted tomography. 1997
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #5: ground glass opacities on Chest CT
Bilateral asymmetrical
areas of Ground
glass opacities in
peripheral
distribution.
C – axial chest CT. Lung window
Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical Imaging. 5th edition
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Differential diagnosis
• Focus on clinical and radiologic findings, and
additional work up, the differential diagnosis
includes:
o Churg-Strauss Syndrome (CSS).
o Bronchiolitis obliterans organizing pneumonia (BOOP).
o Simple pulmonary eosinophilia (SPE)
All of these diseases have eosinophilia as a
remarkable finding.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Churg‐Strauss syndrome
(CSS)
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CCS: Presentation
Churg-Strauss Syndrome:
• Clinically:
o Extrapulmonary involvement: Neurological, paranasal sinus, cardiac
and skin.
o Previous history of asthma.
• Perinuclear - Anti-neutrophil cytoplasmic
antibodies (P-ANCA) is found positive in 48 -73%
of patients.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CSS: Bilateral opacities and enlarged heart size on CXR
• Bilateral opacities in
both lower lobes and
enlarged heart size
which was proved to
be pericardial effusion
on CT.
Yookyung Kim, et al. The spectrum of eosinophilic lung disease: radiologic findings. Journal of computer assisted tomography. 1997
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CSS: consolidation, ground glass opacities, pleural and pericardial effusions on Chest CT
•
•
C – axial chest CT. Lung window
Characteristics of
eosinophilic lung
involvement are Lung
consolidation in the right
lung and Ground glass
opacities in the left side.
Bilateral Pleural effusions
and pericardial effusion.
Subsequent to
cardiomyopathy.
Mason: Murray and Nadelʹs Textbook of Respiratory Medicine, 5th ed.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
CSS: Multiple centrilobular nodules, bronchial wall thickening and ground glass opacities on Chest CT
• Other common
findings are multiple
centrilobular
nodules, bronchial
wall thickening. Also
some areas of diffuse
ground glass
opacities are
present.
C – axial chest CT. Lung window
Yeon J, et al. Eosinophilic Lung Disease: A clinical, radiolic and pathologic overview, RadioGraphics. 2007
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Bronchiolitis obliterans
organizing pneumonia
(BOOP)
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Andres Miranda
Gillian Lieberman, MD
August, 2011
BOOP: Presentation and laboratory
• Histological diagnosis of organizing pneumonia of
unknown etiology.
• Clinical:
o Short history of cough, dyspnea, fever, malaise and weight loss.
• Excellent response to corticosteroid treatment, but
relapses are common.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
BOOP: Relapsing consolidations
Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical Imaging. 5th edition
Multifocal consolidation in upper and mid zones, the relapse is present after stopping the treatment, a suggestive characteristic of this condition.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
BOOP: consolidation and ground glass opacities on Chest CT
• areas of
consolidation in
both upper
lobes, with
associated
ground-glass
opacification.
C – axial chest CT. Lung window
Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical Imaging. 5th edition
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Andres Miranda
Gillian Lieberman, MD
August, 2011
BOOP: consolidation and ground glass opacities affecting both lungs.
• These
consolidations
and groundglass
opacification.
Could be
present in all
lobes.
C – axial chest CT. Lung window
Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical Imaging. 5th edition
32
Andres Miranda
Gillian Lieberman, MD
August, 2011
Simple pulmonary
eosinophilia
(Loeffler Syndrome)
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Simple pulmonary eosinophilia: Presentation
• It is due to the passage of some parasites,
especially nematodes, through the lung.
• Could be asymptomatic or just general symptoms.
• The most common causes are: Ascaris
lumbricoides, Strongiloides Stercoralis and
Toxocara canis.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Simple pulmonary eosinophilia: Migratory opacities.
• Migratory
opacities with
peripheral
distribution.
Sharma Girish. Loeffler syndrome. Medscape reference
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Simple pulmonary eosinophilia: Ground glass
opacities on chest CT
C – axial chest CT. Lung window
• Mixed bilateral areas of consolidation and ground
glass opacities. Usually these changes resolve
spontaneously within one month.
Yeon J, et al. Eosinophilic Lung Disease: A clinical, radiolic and pathologic overview, RadioGraphics. 2007
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Andres Miranda
Gillian Lieberman, MD
August, 2011
SPE: Other findings on CT
• This lesion could be
a common finding
in patient with
Loeffler syndrome,
and can be
concerning about
malignancy,
however in this
case it dissapear
Pulmonary completely at
nodule
C – axial chest CT. Lung window
follow-up.
Yeon J, et al. Eosinophilic Lung Disease: A clinical, radiolic and pathologic overview, RadioGraphics. 2007
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Going back to our patient:
Additional work‐up
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Hospital admission
Our patient was admitted to the hospital for In-patient work-up,
where she underwent:
• Bronchoalveolar lavage (BAL): Red blood cells: 0 Polys: 11,
Lymphs: 6, Monos: 0, Eos: 44, Macro: 39.
•
ABG: Hypoxemia.
•
Bronchoscopy: Negative for anatomic abnormality.
•
Parasites serology: Negative.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: Diagnostic
tests
• Lung biopsy: fibrosis and scarring, increased
number of eosinophiles. Involves bronchial walls,
changes suggestive of pulmonary hypertension.
• P-ANCA: Negatives.
• Control CBC showed WBC: 10,000 with 19%
eosinophils(2000). ESR: 71
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: more diagnostic tests
• Total IgE: 4916.
• Allergens skin testing: Panelevation of
enviromental allergens, including Aspergillus.
• Spirometry: Severe restrictive pattern.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: fibrosis and ground glass opacities on Chest CT
Areas of fibrosis in both upper lobes with subpleural
distributions. Bilateral Ground glass opacities
C – axial chest CT. Lung window
PACS, BIDMC
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: fibrosis and loss of volume on chest CT
• Bilateral peripheral
fibrosis, along with
signs of right upper
lobe loss of volume.
C – coronal chest CT. Lung window
PACS, BIDMC
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Our patient: bronchiectasis, mucus impactation
and centrilobular nodules on chest CT
C – axial chest CT. Lung window
• Bilateral bronchiectasis radiating from perihilar areas.
• Zone of mucus impactation, within bronchiectasis.
• Centrilobular nodules.
PACS, BIDMC
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Allergic Bronchopulmonary
Aspergillosis
A new diagnosis to take into account in our patient.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Presentation
•
Patient with previous history of asthma.
•
There are essential findings for the diagnosis:
•
o
Skin test reactivity to Aspergillus sp.
o
Total IgE level more than 1000 U/L.
o
IgE or IgG against Aspergillus sp. In the blood.
o
Peripheral blood eosinophilia.
Symptoms are general and there are no specific clinical
findings other than these of asthma.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #6: opacities and dilated bronchi
on CXR
• Opacities in both upper lung zones and round opacity in left lower lung zone. Also dilated bronchi in right side are present.
Yookyung Kim, et al. The spectrum of eosinophilic lung disease: radiologic findings. Journal of computer assisted tomography. 1997
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Companion patient #7: Mucus plugging and
bronchiectasis on Chest CT
Bilateral zones of mucous plugging. C – axial chest CT. Lung window
Tubular and cystic bronchiectasis
Goldman: Goldmanʹs Cecil Medicine, 24th ed.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
These findings are almost pathognomonic of Allergic
Bronchopulmonary Aspergillosis in patients with previous history
of asthma, elevated eosinophil count and high serum IgE
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Patient analysis
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Outcome
The diagnosis of Chronic eosinphilic pneumonia and
Allergic Bronchopulmonary Aspergillosis was made
based on the following:
• Clinical findings:
o Long history of respiratory and general symptoms.
o Non clear PMH positive for asthma.
• Laboratory:
o
o
o
o
o
o
Peripheral eosinophilia greater than 1.000 /uL.
Persistent elevated ESR.
IgE level higher than 1.000 U/L.
Severe restrictive pattern on lung function test.
BAL and byopsia: Market eosinophilia and inflammatory changes.
Exclusion of other possible causes: Infection, vasculitis.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Summary of imaging findings
• Chest X-ray:
o Parenchimal densities more accentuated in upper lobes.
o Generalized linear opacities.
o Peripheral pleural thckening.
• Chest CT:
o Areas of peropheral fibrosis and bilateral ground glass opacities
predominant in upper lobes.
o Bilateral bronchiectasis and mucous impactation.
o Centrilobular nodules.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
After the diagnosis was made, the therapy was optimized and
patient has been doing well with symptoms last years; however
his findings on pulmonary imaging have not improved much,
because the patient is in the fibrotic phase of the disease,
which is irreversible.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Conclusions
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Conclusions
•
There are a big number of conditions that can cause
peripheral eosinophilia along with pulmonary symptoms, in
these cases extended work-up will be necessary in order to
make a right diagnosis and provide correct treatment.
•
Different modalities of imaging, such as chest X-ray and CT,
play an important role in the diagnosis of these diseases.
•
The approach bases on radiologic and clinical findings usually
is enough and invasive procedures, as byopsies could be
avoided.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
References
•
•
•
•
•
•
•
•
•
•
•
•
Jeong Yeon Joo , Kun-il Kim, Im Jeong Seo, Chang Hun Lee, Ki Nam Lee, Ki Nam Kim, et al. Eosinophilic
Lung Diseases: A clinical Radiologic, and Pathologic overview. Radiographics. 2007; 27: 617-639.
Mendes Luiz, Luiz Fernando Ferreira. Pulmonary eosinophilia. The Jornal Brasileiro de Pneumologia. 2009;
35(6): 561-573
Cottin Vincent, Jean-Francois Cordier. Eosinophilic pneumonias. Allergy. 2005; 60: 841-857.
Franquet Tomas, Nestor L Muller, Ana Gimenez, Pedro Guembe, Jesus de la Torre, S. Bague.
Radiographics. 2001; 21: 825-837.
Marchand Eric, Jean-Francois Cordier. Idiopathic chronic eosinophilic pneumonia. Orphanet journal of
Rare diseases. 2006, 1:11.
Yookyung Kim, Kyung Soo Lee, Dong-Chull Choi, Steven L Primack, Jung-Gi Im. The spectrum of
eosinophilic lung disease: radiologic findings. Journal of computer assisted tomography. 1997; 21(6): 920930.
Klion Amy D, Peter F Weller. Causes of pulmonary eosinophilia. 2011. UpToDate online.
King Talmadge Jr, Kevin K Brown. Treatment of idiopathic acute eosinophilic. 2011. UpToDate onlie.
Goldman. Goldman’s Cecil Medicine. 24th edition. New York: Elsevier Saunders. 2011. URL:
http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books/. Accesed 08/22/2011
Mason: Murray and Nadel's. Textbook of Respiratory Medicine. 5th edition. New York: Elsevier Saunders.
2010. URL: http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books/. Accesed 08/22/2011.
Adam. Grainger & Allison's Diagnostic Radiology A Textbook of Medical Imaging. 5th edition. Elsevier
Churchill livingstone. 2008. URL: http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books. Accesed
08/22/2011.
Sharma Girish. Loeffler syndrome. Medscape reference. URL:
http://emedicine.medscape.com/article/1002606-overview. Accesed 08/22/2011.
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Andres Miranda
Gillian Lieberman, MD
August, 2011
Acknowledgements
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Andres Miranda
Gillian Lieberman, MD
August, 2011
I would like to thank…
•
Dr. Alexander Bankier.
•
Dr. Ammar Sarwar.
•
Dr. Javier Perez.
•
Dr. Gillian Lieberman.
•
Emiliy Hanson.
•
Classmates of Harvard Medical School.
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