chronic eosinophilic pneumonia and allergic...

Chronic Chronic EosinophilicEosinophilic

Pneumonia and Pneumonia and Allergic Allergic BronchopulmonaryBronchopulmonary

AspergillosisAspergillosis: Two : Two eosinophiliceosinophilic

lung lung diseases in one patient.diseases in one patient.

Andrés F. MirandaUniversidad de Antioquia, Faculty of Medicine

Gillian Lieberman, MD.

Andres MirandaGillian Lieberman, MDAugust, 2011


AgendaAgenda• 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.2


Index Index patientpatient presentationpresentation..

3


OurOur

patientpatient: : HistoryHistory

ofof

presentpresent

illnessillness• 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.

4


OurOur

patientpatient: : ReviewReview

ofof

systemssystems• 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

5


OurOur

patientpatient: : PastPast

medical medical historyhistory• 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.6


OurOur

patientpatient: : PhysicalPhysical

examinationexamination• 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.

7


OurOur patientpatient: : InitialInitial

WorkWork‐‐upup

• 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.

8


OurOur

patientpatient: Pleural : Pleural thickeningthickening, , consoloditationconsoloditation

andand reticulonodularreticulonodular

patternpattern

onon

InitialInitial

PA CXRPA CXR

9

• Bilateral zones

of pleural

thickening and

consolidation,

predominant in

the periphery.

PACS, BIDMC

Furthermore a

diffuse

reticulonodular

pattern is present.


OurOur patientpatient: : parenchimalparenchimal

densitiesdensities InitialInitial

CXR lateralCXR lateral

• Diffuse parenchimal densities more accentuated in upper lobes.

10PACS, BIDMC


• What is the differential diagnosis of a patient with history of general and chronic respiratory symptoms, marked peripheral eosininophilia and diffuse findings on CXR ?

11


EosinophilicEosinophilic Lung Lung DiseasesDiseases

12


13


ChronicChronic EosinophilicEosinophilic

PneumoniaPneumonia (CEP)(CEP)

14


CEP: CEP: PresentationPresentation• 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.

15


CEP: CEP: DiagnosticDiagnostic studiesstudies

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.

16


CompanionCompanion

patientpatient

#1: #1: UpperUpper

lobeslobes

consolidationconsolidation

onon

CXRCXR

17

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

Companion patient #2: migratory infiltrates on CXRCompanion patient #2: migratory infiltrates on CXR

Migratory pulmonary infiltrates, zones of consolidation distributed peripherally.

18Mendes L, et al. Pulmonary eosinophilia, The Jornal Brasileiro de

Pneumologia. 2009



CompanionCompanion

patientpatient

#3: #3: ““PhotographicPhotographic negativenegative

ofof

pulmonarypulmonary

edemaedema””

CXRCXR

19

The peripheral distribution of abnormalities results in the typical finding of “Photographic negative of pulmonary

edema.”

UpToDate, 2011


Companion patient #4: Companion patient #4: subpleuralsubpleural

consolidation and consolidation and pleural effusion on Chest CTpleural effusion on Chest CT

Other common findings

are subpleural

areas of

consolidation in both

lungs.

Right pleural effusion is

also seen, but is

observed in less than

10%.

20

Yookyung

Kim, et al. The spectrum of eosinophilic

lung disease:

radiologic findings. Journal of computer assisted tomography. 1997

C – axial chest CT. Lung window


Companion patient #5: ground glass opacities on Companion patient #5: ground glass opacities on Chest CTChest CT

Bilateral asymmetrical areas of Ground glass opacities in peripheral distribution.

21

Adam. Grainger & Allisonʹs Diagnostic Radiology A

Textbook of Medical Imaging. 5th

edition



Differential diagnosisDifferential 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.

22


ChurgChurg‐‐Strauss syndromeStrauss syndrome (CSS)(CSS)

23


CCS: PresentationCCS: PresentationChurg-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.

24


CSS: Bilateral opacities and CSS: Bilateral opacities and enlarged heart size on CXRenlarged heart size on CXR

• Bilateral opacities in both lower lobes and enlarged heart size which was proved to be pericardial effusion on CT.

25Yookyung


lung disease: radiologic

findings. Journal of computer assisted tomography. 1997


CSS: consolidation, ground glass opacities, pleural CSS: consolidation, ground glass opacities, pleural and pericardial effusions on Chest CTand pericardial effusions on Chest CT

• 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.

26

Mason: Murray and Nadelʹs Textbook of Respiratory

Medicine,

5th ed.



CSS: Multiple CSS: Multiple centrilobularcentrilobular

nodules, bronchial wall nodules, bronchial wall thickening and ground glass opacities on Chest CTthickening 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.

27Yeon



and




28

BronchiolitisBronchiolitis obliteransobliterans

organizing pneumoniaorganizing pneumonia (BOOP)(BOOP)


BOOP: Presentation and laboratoryBOOP: 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.

29


BOOP: Relapsing consolidationsBOOP: Relapsing consolidations

30

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.


BOOP: consolidation and ground glass opacities BOOP: consolidation and ground glass opacities on Chest CTon Chest CT

• areas of consolidation in both upper lobes, with associated ground-glass opacification.

31Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical

Imaging. 5th

edition



BOOP: consolidation and ground glass opacities BOOP: consolidation and ground glass opacities affecting both lungs.affecting both lungs.

• These consolidations and ground- glass opacification. Could be present in all lobes.

32Adam. Grainger & Allisonʹs Diagnostic Radiology A Textbook of Medical

Imaging. 5th edition



Simple Simple pulmonarypulmonary eosinophiliaeosinophilia

((LoefflerLoeffler SyndromeSyndrome))

33


Simple Simple pulmonarypulmonary

eosinophiliaeosinophilia: : PresentationPresentation

• 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.

34



eosinophiliaeosinophilia: : MigratoryMigratory

opacitiesopacities..

• Migratory opacities with peripheral distribution.

35

Sharma Girish. Loeffler

syndrome. Medscape

reference



eosinophiliaeosinophilia: : GroundGround

glassglass opacitiesopacities

onon

chestchest

CTCT

• Mixed bilateral areas of consolidation and ground glass opacities. Usually these changes resolve spontaneously within one month.

36


Yeon



and



SPE: SPE: OtherOther findingsfindings

onon

CTCT

• This lesion could be a common finding in patient with Loeffler syndrome, and can be concerning about malignancy, however in this case it dissapear completely at follow-up.

37

Pulmonary

nodule

Yeon



and




Going back to our patient:Going back to our patient: Additional workAdditional work‐‐upup

38


OurOur patientpatient: Hospital : Hospital

admissionadmissionOur 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.

39


OurOur patientpatient: : DiagnosticDiagnostic

teststests• 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

40


OurOur patientpatient: more : more

diagnosticdiagnostic teststests

• Total IgE: 4916.

• Allergens skin testing: Panelevation of enviromental allergens, including Aspergillus.

• Spirometry: Severe restrictive pattern.

41

Our patient: fibrosis and ground glass Our patient: fibrosis and ground glass opacities on Chest CTopacities on Chest CT

42


Areas of fibrosis in

both upper lobes

with subpleural

distributions.

Bilateral Ground

glass opacities

PACS, BIDMC


Our patient: fibrosis and loss of Our patient: fibrosis and loss of volume on chest CTvolume on chest CT

• Bilateral peripheral fibrosis, along with signs of right upper lobe loss of volume.

43


PACS, BIDMC

C –

coronal chest CT. Lung window

Our patient: Our patient: bronchiectasisbronchiectasis, mucus , mucus impactationimpactation and and centrilobularcentrilobular

nodules on chest CTnodules on chest CT

• Bilateral bronchiectasis radiating from perihilar areas.• Zone of mucus impactation, within bronchiectasis.• Centrilobular nodules.

44


PACS, BIDMC



Allergic Allergic BronchopulmonaryBronchopulmonary

AspergillosisAspergillosisA new diagnosis to take into account in our patient.

45


PresentationPresentation• 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.

46


CompanionCompanion

patientpatient

#6: #6: opacitiesopacities

andand

dilateddilated

bronchibronchi onon

CXRCXR

• Opacities in both upper lung zones and round opacity in left

lower lung zone. Also dilated bronchi in right side are

present.

47Yookyung


lung disease: radiologic

findings. Journal of computer assisted tomography. 1997


CompanionCompanion

patientpatient

#7: Mucus #7: Mucus pluggingplugging

andand bronchiectasisbronchiectasis

onon

ChestChest

CTCT

48Goldman: Goldmanʹs Cecil Medicine,

24th ed.

Tubular

and cystic

bronchiectasis

Bilateral zones of

mucous plugging.



These findings are almost pathognomonic of Allergic

Bronchopulmonary Aspergillosis in patients with previous history

of asthma, elevated eosinophil count and high serum IgE

49


Patient analysisPatient analysis

50


OutcomeOutcomeThe 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 Peripheral eosinophilia greater than 1.000 /uL.o Persistent elevated ESR.o IgE level higher than 1.000 U/L.o Severe restrictive pattern on lung function test.o BAL and byopsia: Market eosinophilia and inflammatory changes.o Exclusion of other possible causes: Infection, vasculitis.

51


Summary of imaging Summary of imaging findingsfindings

• 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.

52


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.

53

ConclusionsConclusions

54



ConclusionsConclusions• 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.

55

ReferencesReferences• 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): 920- 930.

• 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.

56


http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books/

http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books/

http://www.mdconsult.com.libaccess.lib.mcmaster.ca/books

http://emedicine.medscape.com/article/1002606-overview

AcknowledgementsAcknowledgements

57


I would like to thankI would like to thank……• Dr. Alexander Bankier.

• Dr. Ammar Sarwar.

• Dr. Javier Perez.

• Dr. Gillian Lieberman.

• Emiliy Hanson.

• Classmates of Harvard Medical School.

58


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