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Respiratory (04)
Pathology of Pulmonary
Vascular Diseases
January 12, 2015
Dr !eiss
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"ormal arteries
ELASTIN STAINELASTIN STAIN
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Pulmonary #ascular
$iseases Pulmonary e$ema
Pulmonary em%olism an$infarction
Pulmonary hypertension
Di&use pulmonary hemorrhagicsyn$romes
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emo$ynamic pulmonarye$ema
+tiologies increase$ hy$rostatic pressure
leftsi$e$ heart failure(car$iogenic pulmonary e$ema)
other (uncommon)
$ecrease$ oncotic pressure(uncommon cause of pulmonary
e$ema)
orphology acute' congestion
septal (interstitial) e$ema
al#eolar e$ema (transu$ate) al#eolar microhemorrhages
chronic' %ro-n in$uration(.heartfailure cells/ %rosis)
omplications
impaire$ respiratory function
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+$ema $ue to micro#ascular in*ury
3ocal' pneumonia
Di&use'
acute (a$ult) respiratory $istress syn$rome
(RD)
ynonym' noncar$iogenic pulmonary e$ema haracteristics
a%rupt onset
se#ere hypo6emia
$i&use pulmonary inltrates
a%sence of heart failure or 7ui$ o#erloa$
lassication (%ase$ on Pa8293:82ratio)
mil$
mo$erate
se#ere
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RD +tiologies Direct in*ury to lungs
$i&use pneumonia (esp #iral)
aspiration of gastric contents
inhalation' to6ic gases, smo;e, other irritants
near$ro-ning
o6ygen to6icity9prolonge$ mechanical #entilation ra$iation
ystemic $isor$ers
SEPSIS
trauma9%urns meta%olic $isor$ers (eg, pancreatitis, uremia)
$rugs (esp cytoto6ic)
8< D=+ >8 "? =+ (.8< @="!/)
orphology' $i&use al#eolar $amage (DD)
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>he alpha%et soup of shoc;
ystemic acute in7ammatory syn$rome (:R)
Disseminate$ intra#ascular coagulation (D:)
Di&use al#eolar $amage (DD) cute respiratory $istress syn$rome (RD,
.shoc; lung/)
cute tu%ular necrosis (>") cute renal failure (R3)
ultiple organ failure (83),
a;a ultiple organ $ysfunction syn$rome (8D)
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Diffuse alveolar
damage (DAD)
Pathogenesis:
diffuse damage to the
alveolar-capillary
membrane
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Di&use al#eolar $amage (DD)Pathogenesis' $i&use $amage to al#eolarcapillary mem%rane
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orp o ogy o 'Di&use al#eolar $amage
(DD) !ross
hea#y (up to 56 normal-eight)
re$
rm
essentially airless
icroscopic
e6u$ati#e phase
congestion, e$ema,in7ammation
hyaline mem%ranes(%lac; arro-s)
e6u$e$ plasma proteins
necrotic epithelialcell $e%ris
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Di&use al#eolar$amage (DD)
Resolution
granulation tissueformation
granulation tissueresolution
return to normalarchitecture
AND/OR
Proliferati#e phase
type :: pneumocyte
hyperplasia(%lue arro-s)
%rosis (organiAation) intraal#eolar
interstitial (%lac;arro-s)
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RD (clinical, ra$iologic)
Respiratory insuBciency ($yspnea,tachypnea)
e#ere arterial hypo6emia(cyanosis), refractoryto 82 therapy
Ra$iograph' $i&use %ilateralinltrates e6tensi#e
opacication (.-hiteout/)
Decrease$ lung compliance
omplications($ue to hypo6ia) multisystem organ failure
$eath
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DD' outcome Resolution AND/OR
3i%rosis (organiAation) interstitial %rosis
C .honeycom%ing/
>reatment
un$erlying $isease
supporti#e care
Prognosis
sur#i#al' E0F causes of $eath
sepsis
multiorgan failure
$irect lung in*ury
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cute interstitial pneumonia (:P) ynonym' ammanRich syn$rome
Denition' i$iopathic DD
presents in proliferati#ephase
often progressesto organiAation
cute respiratory failure( antece$ent upper
respiratory infection)
Prognosis G5H5F fatal, usually
-ithin 12 months
sur#i#ors
resi$ual chronic lung $isease (interstitial %rosis)
recurrences
SADDLE EMBOLUS
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Pulmonary throm%oem%olism @arge#essel pulmonary throm%osis is rare
8rigin of em%oli
$eep #eins, lo-er e6tremity (I5F) pel#ic #eins
Pre$isposition immo%ility
hypercoagula%le state (throm%ophilia)
8utcome clinical silence' E00F of cases
KE0F compromise su$$en $eath (5F of cases)
acute respiratory compromise
acute cor pulmonale
pulmonary infarction (10F of cases)
recurrence' common (G0F of cases)
pulmonary hypertension(multiple em%oli o#er time)
passage to systemic circulation(para$o6ical em%olism)
SADDLE EMBOLUS
Pulmonary
ACUTE THROMBOEMBOLUS
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Pulmonarythrom%oem%olism
@arge#essel pulmonary throm%osis rare
8rigin of em%oli
$eep #eins, lo-er e6tremity (I5F) pel#ic #eins
Pre$isposition immo%ility
hypercoagula%le state (throm%ophilia)
8utcome clinical silence' E00F of cases
KE0F compromise su$$en $eath
pulmonary infarction (10F of cases)
recurrence' common (G0F of cases)
pulmonary hypertension(multiple em%oli o#er time)
passage to systemic circulation (para$o6ical em%ol(para$o6ical em%olism)
#ia car$iac septal $efect
rare
ELASTIC STAIN
ORGANIZED THROMBOEMBOLUS
ACUTE THROMBOEMBOLUS
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l i l h i ( )
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1 Pulmonary arterial hypertension (P)
:$iopathic (primary) P
erita%le (familial) P
mutations in %one morphogenetic proteinreceptor type 2 (NPR2) signaling path-ay
normal' inhi%ition of #ascular + an$ $ysfunction an$ proliferation
mutation' intimal, me$ial thic;ening
mo$ier genes9en#ironmental triggers
other mutations
Drug an$ to6inin$uce$ P, eg'
fen7uoramine9phenteramine (fen phen)
methamphetamine P associate$ -ith, eg'
connecti#e tissue $iseases (esp systemic sclerosis)
congenital heart $iseases
schistosomiasis
persistent P of the ne-%orn (PP")
PULMONARY ARTERIAL HYPERTENSIONPULMONARY ARTERIAL
HYPERTENSION
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Pulmonary hypertension (P)
2 P $ue to left heart $isease
O systolic or $iastolic $ysfunction
left atrial pressure
pulmonary #enous pressure
pulmonary arterial pressure (P)O #al#ular $isease,
eg mitral stenosis (left atrial pressure)
G P associate$ -ith lung$iseases9hypo6emia
O chronic o%structi#e lung $isease (8PD)
O chronic restricti#e lung $isease
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Pulmonary hypertension (P)
4 hronic throm%oem%olic Pfunctional crosssectional area
of pulmonary #ascular %e$
in pulmonary #ascular
resistancepulmonary hypertension
5 P $ue to unclear mechanisms (miscellaneous)
O hematologic $isor$ers
O systemic $isor$ers
O meta%olic $isor$ers
O others
Pulmonary hypertension'
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Pulmonary hypertension'morphology
ain pulmonary arteries' atherosclerosis
2000 by Radioo!i"a So"i#$y o% No&$' A(#&i"a)&a*i#& A A #$ a+ Radio!&a,'i"- 2000.20/1342
P l h t i h l
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Pulmonary hypertension' morphology(muscular arteries, arterioles)
Low grade (potentially reversible)
intimal thic;ening %rosis me$ial hypertrophy
OT
NORMAL
H5E STAIN.
REMAINING SLIDES
STAINED )OR ELASTIN
6BLAC78
P l h i
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Pulmonary hypertension'morphology
(muscular arteries, arterioles)
ig! grade (irreversible)
ple6ogenic pulmonaryarteriopathy
ple6iform lesions
angiomatoi$ lesions %rinoi$ necrosis
P l h t i
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Pulmonary hypertension' course :$iopathic an$ herita%le P
respiratory $istress
cyanosis right #entricular
hypertrophy fatal cor pulmonale
(0F -ithin 25 yrs)
treatment
current
O #aso$ilators
O heartlung transplant
future' gene therapy(herita%le P)
8ther forms of P'course an$
treatment #ary -ith
un$erlying $isease
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Di&use pulmonaryhemorrhagic
syn$romes
ynonym' $i&use al#eolarhemorrhage (D)
yn$romes
!oo$pasture syn$rome
:$iopathic pulmonary
hemosi$erosis (:P) Vasculitisassociate$
pulmonary hemorrhage
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syn$rome
+tiology' anti%asementmem%rane anti%o$ies
(autoimmune) +pi$emiology
male pre$ominance
2"DO GRD$eca$e
associate$ -ith renal$isease (RP!")
orphology in7ammatory
$estruction of%asement mem%rane
immuno7uorescence'
linearattern ANTI3BASEMENT MEMBRANE ANTIBODY
:$i hi
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:$iopathicpulmonary
hemosi$erosis(:P) +pi$emiology
Pre$ominantly
young chil$ren $ult cases reporte$
+tiology
"o anti%o$y i$entie$
3a#ora%le response toimmunosuppressi#esin$icates possi%leimmune mechanism
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Vasculitisassociate$pulmonary
hemorrhage ssociate$ con$itions
microscopic polyangiitis (P)(hypersensiti#ity #asculitis)
granulomatous polyangiitis(!P) (Megener)
systemic lupus erythematosus
@esion'neutrophilic capillaritis
Prognosis' $epen$s uponun$erlying con$ition
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Vasculitisassociate$pulmonary hemorrhage
ssociate$ con$itions microscopic polyangiitis
(hypersensiti#ity #asculitis)
!P (Megener )
systemic lupus erythematosus immuno7uorescence'
granular pattern
+' immune comple6es in al#eolar
capillary mem%rane (%lac; arro-)
@esion' neutrophilic capillaritis
Prognosis' $epen$s uponun$erlying con$ition
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Pulmonary #ascular
$iseases Pulmonary e$ema
Pulmonary em%olism an$infarction
Pulmonary hypertension
Di&use pulmonary hemorrhagicsyn$romes
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