Camila Downey,2010

GillianLiebermanMD.
08/30/10 09/26/10

Systemiclupuserythematosus (SLE):
Pleuropulmonary Manifestations
Camila DowneyS.
UniversidaddeChile,SchoolofMedicine,YearVII
GillianLiebermanMD.
HarvardUniversity,SchoolofMedicine
Sept17,2010

Camila Downey,2010
GillianLiebermanMD.

Agenda

Introduction
Pleuralinvolvement
Pulmonaryinvolvement
Airwayinvolvement
Pulmonaryvasculardisease
Infections
PatientPresentation
Summary
References
Acknowledgements

Camila Downey,2010
GillianLiebermanMD.

SLE:Epidemiology
Systemiclupuserythematosus (SLE):
Isachronicautoimmunediseasecharacterizedby
microvascular inflammationwiththegeneration
ofautoantibodies thatcanaffectalmostany
organsystem.
Approximately250,000Americanshavesystemic
lupus.(NationalArthritisDataWorkingGroup).
Itspresentationandcoursearehighlyvariable.

Camila Downey,2010
GillianLiebermanMD.

SLE:Pleuropulmonary involvement
ThemajorityofpatientswithSLEdevelop
pleuralorpulmonarydiseaseinthecourseof
theirillness,diagnosedclinicallyandorby
imagestechniques.
Respiratoryinvolvementismorecommonin
menthaninwomen.
Thepleuraisthemostcommonthoracic
localizationofSLE.

Camila Downey,2010
GillianLiebermanMD.

SLE:Pleuropulmonary
manifestations
Infections

Pleuritis withorwithouteffusion
Upperandlowerairwaysdisease
Acutelupuspneumonitis
Alveolarhemorrhage
Organizingpneumonia
Chronicinterstitiallungdisease

Lymphocyticinterstitialpneumonia
Nonspecificinterstitialpneumonia
Usualinterstitialpneumonia
Desquamative interstitialpneumonia

Respiratorymuscleweakness(Shrinkinglungsyndrome)
Pulmonaryhypertension
Pulmonaryembolism
Mediastinal lymphadenophaty

Crestani B.Therespiratorysysteminconnectivetissuedisrders.Allergy 2005;60:71534.

Camila Downey,2010
GillianLiebermanMD.

Pleuralinvolvement
Pleuralinvolvementmay
beasymptomatic,
althoughpleuritic painis
verycommon.
Clinicallyapparent
effusions havebeen
reportedinupto50%of
patientsandpathological
involvementinautopsyin
upto93%ofpatients.
Typicallyassociatedwith
chestpain,dyspnea,
coughandfever.
Crestani B.Therespiratorysysteminconnectivetissuedisorders.Allergy 2005;60:71534.

Camila Downey,2010
GillianLiebermanMD.

Acutelupuspneumonitis
Lungparenchyma
involvementcanbe
acuteorchronic.
Acutelupus
pneumonitis:Non
specific,maysimulate
infection,pulmonary
embolismorother.
Variabledegreeof
respiratoryimpairment
accompaniedbyfocalor
diffusepulmonary
consolidation.

Crestani B.Therespiratorysysteminconnectivetissuedisorders.Allergy 2005;60:71534.

Camila Downey,2010
GillianLiebermanMD.

Acutepulmonaryhemorrhage
Acutepulmonary
hemorrhage:
Bilaterallunginfiltrates,
rangingfromlimited
groundglassopacitiesto
denseconsolidation.
Consolidationcanbe
diffuseorpatchy.

Crestani B.Therespiratorysysteminconnectivetissuedisorders.Allergy 2005;60:71534

Camila Downey,2010
GillianLiebermanMD.

Pulmonaryfibrosis
Chronicinterstitial
pulmonarydisease:
Pulmonaryfibrosis:
honeycomb changes
withperipheraland
basalpredominance,
linearthickened
interlobularseptae,
groundglassattenuation
andparenchymal bands.

Eun AKimetal.Interstitiallungdiseasesassociatedwithcollagenvasculardiseases:Radiological
andhistopathologic findings.RadioGraphics 2002;22:15165

Camila Downey,2010
GillianLiebermanMD.

Lymphocyticinterstitialpneumonia
Lymphocyticinterstitial
pneumonia(LIP):
Usuallyassociatedwith
Sjogren syndrome.
Groundglassopacity,
poorlydefined
centrilobular nodules,
thickeningof
bronchovascular bundle,
interlobularseptae and
cysticairspaces.
Patchyalveolar
infiltrates.
LawrenceKenneyetal.Lymphocyticinterstitialpneumonitis inawomanwithtangiersdisease.Chest2004;126(4):977S.

Camila Downey,2010
GillianLiebermanMD.

AirwayInvolvement
UncommoninSLE.
Upperairway,glottisandcricoarytenoid joints
seemtobethemostcommonlyinvolvedsites.

Camila Downey,2010
GillianLiebermanMD.

Pulmonaryvasculardisease
Pulmonary
Hypertension:
Presentin514%of
patients.Prevalence
tendtoincreasewith
time.
Fewcasesresultinright
heartfailure.
Diagnosissuspectedon
echocardiographyand
confirmedbycardiac
catheterization.
Image from PACS, BIDMC

Camila Downey,2010
GillianLiebermanMD.

PulmonaryHypertension
Dilatedmainpulmonary
artery,abnormalitiesin
perfusion,heterogeneityof
lungattenuation(mosaic
perfusion).

Images from PACS, BIDMC

Camila Downey,2010
GillianLiebermanMD.

Pulmonaryembolism
Pulmonaryembolism:
Mostlyassociatedwith
Antiphospholipid
syndrome.
Chronicpulmonary
embolismcanleadto
pulmonary
hypertension.

Tasneem A.Lalani etal.ImagingFindingsinSystemicLupusErythematosus.RadioGraphics

2004;24(4):106986

Camila Downey,2010
GillianLiebermanMD.

Pulmonaryinfections
Infectionisamajorcauseofmorbidityand
mortalityinSLE.
50%ofdeathsreportedinsomeseries.
Secondarytoinmunosuppression associated
withSLEitselfandinducedbycorticosteroids
andimmunosuppressants.
Susceptibletousualpathogensand
opportunisticpathogens.
Mycobacterial andNocardial infectionsseem
particularlyimportant

Camila Downey,2010
GillianLiebermanMD.

Mycobacterial andNocardial
infection
Mycobacterial Infection:
Consolidation and cavitation
of left apex.

Nocardial infection: Consolidation

and cavitation of right upper lobe.

Tasneem A.Lalani etal.ImagingFindingsinSystemicLupusErythematosus.RadioGraphics 2004;24(4):106986

Camila Downey,2010
GillianLiebermanMD.

Shrinkinglungsyndrome
Dyspnea associatedwithsmalllungvolumes,
elevatedhemidiaphragms andbibasilar
atelectasis.
Syndromeattributedtodiaphragmatic
dysfunction(myopathy)onthebasisof
demonstrationofdecreasedrespiratory
musclestrength

Camila Downey,2010
GillianLiebermanMD.

Shrinkinglungsyndrome:Images
Elevated
hemidiaphragms,small
lungvolumes,and
bibasilaratelectasis

DavidA.LynchMB.Lungdiseaserelatedtocollagenvasculardisease.JThorac Imaging 2009;

24(4):299309

Camila Downey,2010
GillianLiebermanMD.

Ourpatient
Ourpatient:
32yearsoldfemale.
Pastmedicalhistory:
SLEcoursecomplicatedbynephritis,anemia,
serositis andascites.
Vascularstenosis resultinginfacialedemaand
subclavian steal.
StageIVsacraldecubitus ulcercomplicatedby
osteomyelitis
Gastroesophageal refluxdisease

Camila Downey,2010
GillianLiebermanMD.

ClinicalCase:Pastmedicalhistory
Pastmedicalhistory
ESRDstatuspostfailedrenaltransplantrequiring
explant,currentlyonhemodialysis threetimesa
week.
Multiplehospitalizationsforlinesepsis.
HistoryofMSSAendocarditis complicatedby
embolicstrokeandresultantseizuredisorder.
Sicklecelltrait
Pulmonaryhypertension
Restrictivelungdisease

Camila Downey,2010
GillianLiebermanMD.

ClinicalCase:Actualhistory
Consultsbecauseofhistoryofworsening
shortnessofbreath,worsewhenlyingdown
inlastfewweeks.Patientdeniesanychest
painorcough.
Findingsonphysicalexam:
Febrileupto101
Heartrate:83,Bloodpressure:110/75,Respiratory
rate:16,SpO2:98%on2litersofO2onadmission
Pulmonaryexam:Cracklesatbasesbilaterallyno
increasedworkofbreathing.

Camila Downey,2010
GillianLiebermanMD.

ClinicalCase:Laboratoryexams
Findingsonlaboratoryexams:
Hematocrit:23.2%,repeated:21.8%
Hemoglobin:7.1mg/dL.

Patientwastransfusedoneunitofpackedred
bloodcells,withappropriaterisein
hematocrit andimprovementinshortnessof
breath.

Camila Downey,2010
GillianLiebermanMD.

ClinicalCase:Images
Duringworkup,concerningformultifocal
pneumoniavs.pulmonarycongestionChestX
Ray(CXR)andComputedTomography(CT)
areobtained

Camila Downey,2010
GillianLiebermanMD.

CXRandCoronalCT

Image from PACS, BIDMC

Camila Downey,2010
GillianLiebermanMD.

ChestXRay
Findingsconcerningfor
multifocalpneumonia
thoughacomponentof
vascularcongestion
cannotbeentirely
excluded.
Increasedgroundglass
opacities.
Atelectasis andpleural
effusions.

Camila Downey,2010
GillianLiebermanMD.

ChestCT:Effusions,atelectasis
Bilateralmoderatesized
pleuraleffusions
worsensincethree
monthsago.
Adjacentatelectasis at
lungbases
Increased
heterogeneousair
spacedisease
compatiblewith
multifocalpneumonia.

Camila Downey,2010
GillianLiebermanMD.

ChestCT:Pulmonaryedema
Significantprominence
ofpulmonary
vasculaturewithseptal
thickeningandpatchy
groundglassopacity
bilaterallycompatible
withmoderateto
severepulmonary
edema.

Camila Downey,2010
GillianLiebermanMD.

ChestCT:Pulmonaryhypertension
Massivelydilatedmain
pulmonaryartery
(47mm), enlarged
azygos vein,compatible
withpulmonary
hypertension.

Camila Downey,2010
GillianLiebermanMD.

ChestCT:Cardiomegaly
Cardiomegaly with
enlargedrightatrium
andseptumthickening.

Camila Downey,2010
GillianLiebermanMD.

Summary
SLEischronicautoimmunediseasethatcan
affectalmostanyorgansystem.
MajorityofpatientswithSLEdeveloppleural
orpulmonarydiseaseinthecourseoftheir
illness.
Mostcommonpleuropulmonary
manifestationsofSLEare:

Infections
Pleuritis withorwithouteffusion
Acutelupuspneumonitis
Pulmonaryembolismthatcanleadtopulmonaryhypertension

Camila Downey,2010
GillianLiebermanMD.

References

Crestani B.Therespiratorysysteminconnectivetissuedisorders.Allergy,
2005;60:71534
DavidA.LynchMB.Lungdiseaserelatedtocollagenvasculardisease.J
Thorac Imaging. 2009;24(4):299309
Tasneem A.Lalani etal.ImagingFindingsinSystemicLupus
Erythematosus.RadioGraphics. 2004;24(4):106986
LawrenceKenneyetal.Lymphocyticinterstitialpneumonitis inawoman
withtangiersdisease.Chest.2004;126(4):977S
PegoReigosa etal.Respiratorymanifestationsofsystemiclupus
erythematosus:oldandnewconcepts.Bestpractice&ResearchClinical
Rheumatology.2009;23:46980
Eun AKimetal.Interstitiallungdiseasesassociatedwithcollagenvascular
diseases:Radiologicalandhistopathologic findings.RadioGraphics. 2002;
22:15165
BartelsC.etal.SystemicLupusErythematosus,
http://emedicine.medscape.com/article/332244overview,09/13/2010

Camila Downey,2010
GillianLiebermanMD.

Acknowledgements

GillianLiebermanMD.
CarolRidgeMD.
EmilyHanson
LarryBarbaras

Thankyou