04_ Pathology of Pulmonary Vascular Diseases 01_09_2015.ppt

8/9/2019 04_ Pathology of Pulmonary Vascular Diseases 01_09_2015.ppt

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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

04_ Pathology of Pulmonary Vascular Diseases 01_09_2015.ppt

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