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VASCULITELE

SISTEMICE

Prof. Coman TanasescuSpitalul Colentina

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DEFINIIE

grup heterogen de boli relativ rare

clasificare imprecis

caracterizate de prezena la nivelulperetelui vascular :

infiltrat inflamator

necroz

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MRIME VAS PRIMAR SECUNDAR

ARTERE MARI - ART. CELULE GIGANTE- ART. TAKAYASU

-AORTITA REUMATOID- INFECII LUES, TBC

ARTERE MEDII - POLIARTERITANODOAS CLASIC

BOALA KAWASAKI

- POLIARTERITANODOAS SECUNDARINFECIEI CU VHB

ARTEREMEDII/MICI

- GRANULOMATOZA

WEGENER*

- SDR. CHURG-STRAUSS*

- POLIANGIITA

MICROSCOPIC*

- SECUNDARE

COLAGENOZELOR

(AR, LES, SS)

- MEDICAMENTE

- INFECII (HIV)

VASE MICI

-(arteriole, capilare,

- venule )

-- PURPURA HENOCH-

- SCHONLEIN

-- CRIOGLOBULINEMIA

--VASCULITA CUTANAT- LEUCOCITOCLASTIC

-- INFECIA CU VHC-- MEDICAMENTE

CLASIFICAREA VASCULITELOR SISTEMICE

*ANCA + AFECTARE RENAL RSPUNS BUN CICLOFOSFAMID

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CLASIFICARE PATOGENIC

TIP I IgE Churg-Strauss

TIP IIcitotoxicitate celular Granulomatoza Wegener, poliangiita microscopic,

Kawasaki

TIP III complexe imune circulante PAN, purpura HS, crioglobulinemia, vasculitele

secundare bolilor de colagen

TIP IVhipersensibilitate mediat celular Arterita CG, Takayasu

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INVESTIGAREA VASCULITELOR

Sindromul inflamator

hemoleucograma eozinofileVSH

proteina C reactiv fibrinogen

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INVESTIGAREA VASCULITELOR

Afectarea de organ

Orice biopsie se efectueaz naintea

terapiei de imunosupresie Sumar urin, funcia renal Teste hepatice

Imagistic CT, MR SNP viteze conducere, biopsie Cord (+ eco), tub digestiv

Piele - biopsie

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INVESTIGAREA VASCULITELOR

Serologie

Autoanticorpi

ANCA, ANA

Complement seric, crioglobuline

FR, antiFL

HemoculturiVirusuri VHB, VHC, HIV, CMV

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ANTICORPII ANTICITOPLASM DENEUTROFIL (ANCA)

Descrii n 1982

Cei mai specifici sunt c-ANCA

Asociai cu mecanism tip II

Granulomatoza Wegener

Poliangiita microscopic

Boala Kawasaki

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ANTICORPII ANTICITOPLASM DENEUTROFIL (c-ANCA)

- anti peroxidaza 3 -

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ANTICORPII ANTICITOPLASM DENEUTROFIL (p-ANCA)

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DIAGNOSTIC DIFERENIAL

Aspectul sistemic

Endocardita infecioas, paraneoplaziile

Embolii sistemice

Mixom atrial

Tromboze difuze

SAFL, crioglobulinemii

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VASE MEDII I MICI

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POLIARTERITA

NODOAS

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PAN

Kussmaul i Maier 1866(periarterit!)

Inflamaie artere medii i mici Nu afecteaz arteriole, capilare,

venule

Nu se asociaz cu glomerulonefrita

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PAN

Inflamaie bogat n neutrofile Necroz fibrinoidAnevrisme ale peretelui arterial

Absena granuloamelor

Asociere cu infecia cu VHB

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PAN

Febr, astenie sever,stare general alterat

Artralgii, mialgii

Semne de PAN dupspt. luni

Piele livedo, nodulisubcutanai, gangrene

SNP mononevritamultiplex gambe,brae

Digestiv anginaintestinal, infarctehepatice, colecistit iapendicit acut,perforaii

Renal vasculitavaselor interlobulare,HTA

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PAN

Laborator nespecificinflamaie

Hematurie

microscopic Infecie VHB Imagistic anevrisme

mezenterice -

Tratament:

Imunosupresie Corticoizin puls Cure scurte, intense

Antiviral

lamivudina, entecavir,

NU IFN!!

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LIVEDO RETICULARIS

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Boli asociate cu livedo reticularis

sindromul antifosfolipidic

LES SAFL

poliarterit nodoas

crioglobulinemii

scleroza sistemic

boli infecioase (tuberculoz, sifilis)

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COLECISTIT ACUT NELITIAZIC VASCULITAARTEREI COLECISTICE

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POLIANGIITA MICROSCOPIC

Vase mici arteriole, capilare, venule Mai frecvent dect PAN Confuzii cu PAN!!

Leziuni necrotice cu puine depoziteimune aspectpauci imun

Absena granuloamelor

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POLIANGIITA MICROSCOPIC

Tropism

renal - GN

pulmonarcapilarit sindrom renopulmonar

pielepurpur palpabil

SNP mononevritis multiplex

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GRANULOMATOZA

WEGENER

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GW

Vase mici rar medii Inflamaie granulomatoas Necroze perete vascular

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TUMORA ORBITA STANGA

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NODULI PULMONARI BILATERALI UNII DIN EI SUNTNECROZATI

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HEMORAGIE ALVEOLARA

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PURPURA

HENOCH-SCHONLEIN

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- tetrada caracteristic -

Purpura (purpura reumatic) Artrite

Dureri abdominale Glomerulonefrit

Heberden 1801 Henoch (studentul lui Schonlein)

digestiv i renal

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PURPURA PALPABILA

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DIAGNOSTIC

Biopsie

Excluderea altei boli asociate

Vasculite GW, PAN, poliangiitamicroscopic Colagenoze LES, AR, DM, SS Infecii VHB, VHC, endocardita

infecioas Paraproteinemii crio,

macroglobulinemii

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VASCULITELE DE VASE MARI

ARTERITA CU CELULE GIGANTE

ramificaii de ordinul 2-5 ale aortei artere extracraniale ale capului mai rar aorta

ARTERITA TAKAYASU

aorta i ramurile mari

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histopatologie

infiltrat cu mononucleare (LT, macrofage) panmural, granulomatos

celule multinucleare gigante pot lipsi lamina elastic intern i extern sedistrug hiperplazie intima rspuns imun mediat LT LB neimplicat NU autoanticorpi!! inflamaia sistemic mediat de

interleukine IL-1 i IL-6

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INFILTRAT LIMFO-

PLASMOCITAR

CELULE GIGANTE INCONTACT CU LAMINA

ELASTICA

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STENOZA ARTEREI AXILARE

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TRATAMENT

CORTICOTERAPIE

1mg/kg corp 0,5mg/kg corp

durata luni, ani

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SINDROM

ACG/PMR

ARTERITA CRANIAL

-CEFALEE, SCALP SENSIBIL

- ATROFIE N.OPTIC

-CLAUDICAIE MASETER

-PMR

ACG/AORTIT

-CLAUDICAIE BRA

-ABSENA PULS

-ANEVRISM AORT

-INSUF.AORTIC

-PMR

SINDROM FEBRIL

-FEBRA, FRISON

-TRANSPIRAII NOCT.

-ANOREXIE

-DEPRESIE

PMR IZOLAT

-DURERI CENTURI

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POLIMIALGIA REUMATIC

Simptome bilaterale, simetrice

Redoare la mobilizare

Dureri musculare n centuri scapular,pelvin

Bursita subacromiala

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ALTE VARIANTE ACG/PMR

STARE FEBRIL PRELUNGITCAUZ NECUNOSCUT

ALTERARE DE STARE GENERAL SINDROM INFLAMATOR UMORAL

ANEMIE HIPOCROM CRONIC

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Artere elastice mari aorta, coronarepulmonare

Sindrom de arc aortic

Stenoze, anevrisme

Femeie tnr Inflamaia sistemic uneori discret

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ANEVRISM DE ARC AORTIC

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Arteriografie, angioRM

Pronostic sever

Corticoterapie, aspirina, MTX

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Ulceraii bucale recurente

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Leziuni oculare

Uveit anterioar

Uveit posterioarVasculit retinian

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Afectarea pielii

Eritem nodos

Noduli acneiformi Papulo-pustule

test patergie pozitiv

T t t i iti

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Test patergie pozitiv

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Tromboze venoase profunde

Vena cav

S. Budd-ChiariVen port

Anevrisme arteriale, ruptur aort