Download - vasculite 2014
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Vasculite sistemice
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Proces clinico-patologic caracterizat prin inflamaţia peretelui vascular
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… sediul inflamaţiei
•vase mari/medii/mici
•teritoriul vascular
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… tipul inflamaţiei
•necrotizantă
•granulomatoasă
•leucocitoclazică, etc.
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… mecanismele inflamaţiei
• reacţiile de hipersensibilitate de tip I-IV în diferite asocieri
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… consecinţele inflamaţiei
•ischemia teritoriilor vasculare afectate
•simptome sistemice +
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Clasificare
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•ACR
•Chapel -Hill
•Lie
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Clasificarea Lie
Vasculite primare
Vasculite secundare
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Vasculite primare
1. cu afectarea vaselor mari, medii şi mici
2. cu afectarea vaselor medii şi mici
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Vasculite primare
3. cu afectarea vaselor mici
4. neclasificabile
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Vasculite primare cu afectarea vaselor mari,
medii şi mici
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1. arterita Takayasu
2. arterita cu celule gigante/polimialgia reumatică
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3. angeita izolată a SNC
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Vasculite primare cu afectarea vaselor
medii şi mici
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1. Poliarterita nodoasă
2. Sindromul Churg-Srauss
3. Granulomatoza Wegener
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Vasculite primare cu afectarea vaselor mici
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1. Angeita microscopică
2. Vasculite de hipersensibilitate
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Vasculite primare neclasificabile
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1. Sindromul Behçet
2. Boala Buerger
3. Sindromul Cogan
4. Boala Kawasaki
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Vasculite secundare
De obicei afectează predominent vasele mici
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Vasculite secundare
Infecţii:
VHB,VHC, HIV, parvovirus,CMV, VEB, HTLV
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Vasculite secundare
Infecţii:
Streptococ, stafilococ, salmonela, yersinia
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Vasculite secundare
Infecţii:
Mycobacterii
T.pallidum
Ricketsii
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Vasculite secundare
Boli ţesut conjunctiv:
Boala lupică
Poliartrita reumatoidă
Sindrom Sjogren
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Vasculite secundareNeoplazii:
Limfoame/leucemii
Sindroame mieloproliferative
Sindroame mielodisplazice
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Vasculite secundare
Induse medicamentos
Crioglobulinemii
Vasculita hipocomplementemică
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Vasculite secundare
Urticaria vascularitică
Vasculita posttransplant
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Patogenie •Mecanisme patogenice specifice fiecărei vasculite
•Mecanisme patogenice comune
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Mecanisme specifice
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•Reacţia de hipersensibilitate de tip III
… depunerea de complexe imune/reacţie Arthus
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•Reacţia de hipersensibilitate de tip II
… anticorpi anticelulă endotelială
… ANCA
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Anticorpi anticelulă endotelială
… liză mediată de complement
… ADCC
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Anticorpi anticelulă endotelială
… promovarea trombozei şi a proliferării miointimale
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ANCA
… ADCC… liză mediată de complement
MPO şi PR3 exprimate pe endoteliul vascular
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ANCA
… liză mediată prin PMNPMN
MPO
PR3
ANCA
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… promovarea aderenţei PMN la endoteliu
… activare şi degranulare PMN
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•Reacţia de hipersensibilitate de tip IV
… reacţie tuberculinică/formarea de granuloame
… promovarea apoptozei prin LTC
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Mecanisme comune
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Celula endotelială
activată
Vasculopatie ocluzivă
Promovarea trombozei
Vasospasm
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Promovarea trombozei
… creşterea factorului VII şi PAI 1… expresia moleculelor de adeziune pentru leucocite şi trombocite
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Vasospasm
… creşterea tromboxan şi endotelină 1
… scăderea NO şi PGI2
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Celula endotelială
activată
Eliberare mediatori,
citokine proinflamatorii
şi factori de creştere
Expresie HLA cl.II şi prezentare
antigen
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Tratamentul mecanismelor
patogenice comune
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•antiagregante/anticoagulante
•vasodilatatoare
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Diagnostic
biopsie angiografie
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Angiografia •Segmente stenotice uniforme
lungi ce alternează cu zone cu calibru normal şi anevrisme fuziforme şi saculare
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Angiografia •tromboze
•ocluzii arteriale
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Angiografia
•absenţa sau non-dominanţa plăcilor, neregularităţilor sau ulceraţiilor
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Vasculite primare cu afectarea vaselor mari,
medii şi mici
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Arterita cu celule gigante/polimialgia
reumatică
Asociate în 40-60% cazuri
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Arterita cu celule gigante = arterita Horton = arterita temporală = arterita craniană = arterita granulomatoasă
Definiţie
• pacienţi > 50 ani• ramurile craniene ale aa. emergente din arcul aortic
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Teren genetic - HLA DR4
Etiopatogenie
RI celular tip granulomatos împotriva unor antigene din elastica vasculară sau a unor antigene virale (parvovirus B19, adenovirus, virus sinciţial respirator)
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Preferinţă pentru carotide externe/interne şi ramuri, îndeosebi:
Histopatologie
•artera temporală superficială•artera oftalmică•artere ciliare posterioare•artere vertebrale
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Mai puţin frecvent:
•artera carotidă internă
•artera carotidă externă
•artera centală a retinei
•aorta
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Inflamaţie segmentară a peretelui vascular cu:•necroza peretelui arterial inclusiv lamina elastică•granuloame cu celule gigante multinucleate, limfocite TH1, fibroblaste, eozinofile, rar PMN, LB absente
•tromboza lumenului
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Tablou clinic
•vârsta de debut 50-90 ani
•sex ratio 1/2
•debut brusc sau insidios
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• scădere apetit, astenie, scădere ponderală, febră
• 15% cazuri “ febră de origine necunoscută” la debut - 39 0 C cu frisoane
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•cefaleea = cel mai frecvent simptom prezent la 2/3 bolnavi
•apare precoce
•poate fi primul simptom
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•de obicei intensă, caracter lancinant
• localizată pe regiuni corespondente arterelor inflamate sau difuză
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•hiperestezia scalpului•de obicei asociată cu cefaleea
•de obicei în teritoriul arterelor temporale şi occipitale
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•simptome vizuale
•diplopie
•ptoză
•scăderea acuităţii vizuale până la cecitate
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•simptome vizuale•pot fi simptomul iniţial
•pot fluctua iniţial dar odată stabilit defectul vizual este permanent
•ochiul controlateral este afectat în 1-2 săptămâni dacă nu se începe tratamentul
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Cauze cecitate
•ischemia nervului şi tractului optic secundară vasculitei arterei oftalmice sau a aa. ciliare posterioare
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Cauze cecitate
•rar obstrucţia arteriolelor retiniene
•rar infarct cortex occipital
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Examen FO
nevrita optică ischemică
tardiv atrofie optică
•paloare şi edem al discului optic•exudate vătoase şi microhemoragii
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•claudicaţie intermitentă
muşchii masticatori, limbă, faringe, extremităţi
de obicei la mestecat carne
poate fi mai accentuată unilateral
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•spasm al musculaturii faciale
vasculita arterei faciale
rar, prin obstrucţie vasculară severă
•gangrenă extremităţi, scalp sau limbă
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•Simptome neurologice - 30% cazuri
AIT/stroke în teritoriul carotidian sau vertebro-bazilar
mononeuropatii şi polineuropatii periferice
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•Simptome respiratorii - 10% cazuri
tuse, odinofagie, disfonie
prin ischemie sau hiperiritabiliatatea teritoriilor irigate de arterele inflamate
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•Simptome prin afectarea arterelor mari - 10 - 15% cazuri
claudicaţia membrelor superioare
sufluri la auscultaţia aa. carotide, subclavie, axilară, brahială
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puls scăzut sau absent la braţe sau carotide
fenomene Raynaud
nodozităţi pe artera temporală ce are pulsatilitate redusă
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rar angină, IMA, ICC
Complicaţii tardive
anevrisme aortă toracică
disecţie de aortă
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Polimialgia reumatică - definiţie şi criterii de diagnostic
• durere şi redoare matinală “în centură” şi la nivelul gâtului > 1 lună
• vârsta > 50 ani
• VSH > 40 mm/h
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Polimialgia reumatică
• răspuns rapid la corticoterapie doză mică
• excluderea polimiozitei, poliartritei reumatoide, neoplazie, infecţie cronică
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diagnosticul pozitiv = toate criteriile îndeplinite
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Histopatologie
• leziuni vasculitice identice cu arterita cu celule gigante la nivelul arterelor mari şi mijlocii ce irigă musculatura centurilor, coapselor, feselor şi cefei
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• biopsie musculară normală sau atrofie musculară nespecifică tip II
• sinovită limfocitară la niv. artic. genunchi, umeri, sternoclaviculare, sacroiliace
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Tablou clinic
50% simptome sistemice - febră mică, scădere ponderală
!!! febra mare cu frisoane indică asociere cu arterita cu celule gigante
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atralgiile şi mialgiile pot avea debut acut sau insidios
malaise, fatigabilitate, depresie
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durere şi redoare > astenia musculară
de obicei debut la nivelul centurii scapulare apoi centura pelvină şi muşchii cefei
asociat redoare matinală
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Alte simptome
sindrom de tunel carpian
1/3 sinovită asimetrică şi autolimitată genunchi, RCC şi MCF
tenosinovite
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Paraclinic
anemie normocromă uşoară/moderată
trombocitoză
VSH mult crescut dar ocazional normal
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ANA, FR absente
1/3 anomalii ale testelor hepatice: FA, TGO şi prelungirea timp de protrombină
![Page 85: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/85.jpg)
Biopsia de arteră temporală
Pletismografia oculară - scăderea fluxului sanguin ocular
Angiografia
![Page 86: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/86.jpg)
Criterii de diagnostic pentru arterita cu celule gigante
1.Vârsta la debut 50 ani
2.Cefalee cu debut recent sau modificată recent ca topografie sau caracter
![Page 87: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/87.jpg)
3.sensibilitate la palpare, nodozităţi sau pulsatilitate scăzută a arterei temporale
4.VSH 50 mm/h
5.biopsia arterei temporale ce arată vasculită granulomatoasă cu celule gigante
![Page 88: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/88.jpg)
Diagnosticul pozitiv = 3 criterii
![Page 89: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/89.jpg)
Diagnosticul diferenţial al arteritei cu celule gigante
1. Cu vasculitele ce afectează arterele mici şi mijlocii prin teritoriul vascular diferit, histopatologie şi localizarea visceralizărilor
![Page 90: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/90.jpg)
2. Cu vasculitele ce afectează arterele mari prin vârsta pacientului şi distribuţia leziunilor
3. Cu amiloidoza primară cu afectare vasculară prin biopsie
![Page 91: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/91.jpg)
Diagnosticul diferenţial al polimialgiei reumatice
1. Cu polimiozita: astenia > durerea, enzime musculare, EMG, biopsia musculară
![Page 92: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/92.jpg)
2. Cu poliartrita reumatoidă seronegativă : lipsa predominenţei afectării articulare distale simetrice şi a eroziunilor articulare
![Page 93: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/93.jpg)
3. Cu spondilartropatiile seronegative : HLA B27, afectarea coloanei vertebrale, entesita, uveita
![Page 94: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/94.jpg)
Tratamentul arteritei cu celule gigante
Corticoterapie 40-60 mg/zi
Pulsterapie cu metilprednisolon pentru scădere recentă a acuităţii vizuale
![Page 95: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/95.jpg)
Pentru lipsă răspuns se creşte doza la 80-100 mg/zi
Evaluare răspuns la 2-4 săptămâni: dispariţia simptomelor reversibile, scăderea VSH şi CRP
![Page 96: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/96.jpg)
Pentru răspuns se scade gradat doza cu maxim 10 % din doza totală la 2 săptămâni urmărind simptomele şi markerii inflamaţiei
Corticoterapia la 2 zile are eficienţă scăzută
![Page 97: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/97.jpg)
există tendinţă la autolimitare în 1-2 ani ce permite oprirea corticoterapiei
pentru ineficienţă/toxicitate a corticoterapiei se pot încerca imunosupresoare, D-penicilamina, antimalarice, dapsona
![Page 98: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/98.jpg)
Tratamentul polimialgiei reumatice
Se începe cu AINS
Dacă nu există răspuns în 2-4 săptămâni se administrează 5-20 mg/zi prednison
![Page 99: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/99.jpg)
Vasculite primare cu afectarea vaselor
medii şi mici
![Page 100: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/100.jpg)
Granulomatoza Wegener
![Page 101: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/101.jpg)
leziuni granulomatoase necrotizante tract respirator
superior şi inferior+
glomerulonefrită
vasculită cu afectarea altor viscere+
+
cANCA
![Page 102: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/102.jpg)
•Debut regional ca inflamaţie granulomatoasă necrotizantă
•Vasculita apare în evoluţie
![Page 103: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/103.jpg)
0.4/105/an cazuri noi
Epidemiologie
vârf de incidenţă decada 4-5
uşoară predominenţă masculină
debut predom. în anotimpul rece
![Page 104: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/104.jpg)
Patogenie
![Page 105: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/105.jpg)
antigenul declanşator are poartă de intrare respiratorie şi determină reacţie de hipersensibilitate de tip granulomatos
![Page 106: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/106.jpg)
•în formarea granuloamelor - reacţie de hipersensibilitate de tip IV mediată LTDH CD4 cu profil T helper 1
•vasculita apare în evoluţie
•cANCA sunt secretaţi de ţesutul limfoid pulmonar
![Page 107: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/107.jpg)
Morfopatologie
![Page 108: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/108.jpg)
Leziunile pulmonare
•Granulom cu necroză centrală şi histiocite în palisadă şi fibroblaşti la periferie
•Eozinofilie tisulară în 63% cazuri
![Page 109: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/109.jpg)
•afectarea arterelor, venelor şi capilarelor poate fi prezentă dar vasculita nu este obligatorie pentru diagnostic
![Page 110: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/110.jpg)
Leziunile CRS
•asocierea dintre necroză, inflamaţie granulomatoasă şi vasculită
![Page 111: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/111.jpg)
Leziunile renale
•GN necrotizantă focală
•GN proliferativ-difuză
•nefrită interstiţială
•hialinizare glomeruli/formare semilune
![Page 112: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/112.jpg)
Vasculita•inflamaţie cronică/ granuloame în peretele vascular
•necroză fibrinoidă
•distrucţia muscularei şi a laminei elastice •fără formare de microanevrisme
![Page 113: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/113.jpg)
Tablou clinic
![Page 114: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/114.jpg)
Debut•Simptome CRS
•Sinuzită cronică •Rinită cronică •Ulceraţii nazale •Otită seroasă medie
•Simptome sistemice
![Page 115: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/115.jpg)
Pot apare•Otită supurativă
•Ulceraţii palat
•Mastoidită
•Diminuare auz
![Page 116: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/116.jpg)
Clinic •rinoree mucoasă/purulentă/sanguinolentă
•obstrucţie nazală
•dureri regionale
![Page 117: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/117.jpg)
Rx sinusuri
•îngroşarea mucoasei sinusurilor maxilare
•± distrucţie pereţi osoşi
![Page 118: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/118.jpg)
Afectarea pulmonară - 85% cazuri •tuse
•dispnee
•hemoptizie
•mai rar durere pleuretică sau obstrucţie traheală
![Page 119: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/119.jpg)
Radiografia pulmonară
•Noduli uni- sau bilaterali ± cavitaţi
•Infiltrate pulmonare uni- sau bilaterale
•Mai rar pleurezie sau atelectazie
![Page 120: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/120.jpg)
PFR
•Pattern restrictiv sau obstructiv
![Page 121: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/121.jpg)
Afectarea renală
•20% cazuri la debut
•77% cazuri la 2 ani•proteinurie•hematurie•IR•Rar HTA
![Page 122: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/122.jpg)
Afectarea cutanată - 50% cazuri
•Purpura vasculară - vasculita negranulomatoasă
•Leziuni nodulare
![Page 123: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/123.jpg)
Afectarea oculară - 50% cazuri
•afectarea orbitei secundară sinuzitei purulente
•proptoză
•conjunctivită, episclerită, ulcere corneene, uveită, nevrită optică
![Page 124: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/124.jpg)
Afectarea neurologică - 33% cazuri
•neuropatie periferică/ mononevrita multiplex
•afectarea nn. cranieni II, VI, VII
•convulsii, stroke, cerebrită
•diabet insipid
![Page 125: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/125.jpg)
Afectarea cardiacă
•arterită coronară
•pericardită
•cardiomiopatie dilatativă
![Page 126: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/126.jpg)
Afectarea musculo-scheletală
•artralgii - 70% cazuri; precoce; fără eroziuni
•mai rar artrită
![Page 127: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/127.jpg)
Paraclinic
![Page 128: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/128.jpg)
Hematologic
•anemie normocormă normocitară•leucocitoză fără creşterea Eo
•trombocitoză
![Page 129: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/129.jpg)
Sindrom inflamator•VSH crescut
•CRP - marker de urmărire evoluţie
•hipergamaglulinemie cu creşterea îndeosebi a IgE
•complementul normal sau crescut
![Page 130: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/130.jpg)
Serologic•FR prezent în 50% cazuri•ANA negativ
•cANCA - 90% cazuri; specificitate anti PR3; pot persista după tratament
•crioglobuline absente
![Page 131: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/131.jpg)
Diagnostic
![Page 132: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/132.jpg)
clinic •afectarea CRS•proptoza •infiltrate pulmonare •anomalii sediment urinar
cANCA
biopsie inflamaţie granulomatoasă
![Page 133: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/133.jpg)
Tratament
![Page 134: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/134.jpg)
Ciclofosfamida •2 mg/kc/zi per os
•puls-terapia nu are avantaje asupra administrării po •în asociere cu corticoterapie
Ciclosporina
![Page 135: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/135.jpg)
Prognostic
![Page 136: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/136.jpg)
Afectarea renală - cel mai important factor de prognostic în primul an de la diagnostic
Afectarea pulmonară - cel mai important factor de prognostic în evoluţie
![Page 137: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/137.jpg)
Poliarterita nodoasă
![Page 138: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/138.jpg)
Caracteristici
Afectarea frecventă a:
•tegumentelor •articulaţiilor •nervilor periferici•intestinului•rinichiului•cordului
![Page 139: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/139.jpg)
Caracteristici
Afectarea rară a: •arterei pulmonare
•splinei
![Page 140: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/140.jpg)
Caracteristici
Poate apare ca o complicaţie a:
•sindromului Sjogren
•poliartritei reumatoide
•infecţiei cu virus hepatitic B sau C
![Page 141: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/141.jpg)
Epidemiologie
sex ratio 2/1 poate apare la copii
Incidenţă
•77/106 în populaţia cu endemie de hepatită B
•4.6 - 9/106 în populaţia generală
40-60 ani
![Page 142: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/142.jpg)
Histopatologie
•artere musculare mici şi medii
•predilecţie pentru zona de bifurcare
•alternanţă leziuni inflamatorii cu zone de ţesut normal şi cu zone cicatriciale
![Page 143: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/143.jpg)
Leziuni inflamatorii
•panmurale
•focale
•necrotizante
![Page 144: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/144.jpg)
Leziuni inflamatorii
•Infiltrat celular pleomorf
•Necroză fibrinoidă
•predom. PMN•nr. variabil de limfocite şi eozinofile
![Page 145: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/145.jpg)
•Tromboză
•Arhitectura vasului, inclusiv lamina elastică, complet compromisă
•Dilataţii anevrismale
![Page 146: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/146.jpg)
Leziunile vindecate
•proliferare ţesut fibros şi celule endoteliale cu obliterare lumen
![Page 147: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/147.jpg)
Tablou clinic
![Page 148: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/148.jpg)
Simptome sistemice
•purpura palpabilăLeziuni cutanate - 50% cazuri
•ulceraţii/necroză ischemică
•livedo reticularis
•noduli
![Page 149: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/149.jpg)
•Poliartrită asimetrică nedeformantă cu afectarea artic. mari de la nivelul membrului inferior
Artralgii/artrite - 50% cazuri
![Page 150: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/150.jpg)
•poate fi simptom de debut
Neuropatia periferică - 50 - 70% cazuri
•extremităţile superioare şi inferioare sunt afectate cu aceeaşi frecvenţă
![Page 151: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/151.jpg)
debut acut
Neuropatia periferică
•durere şi parestezii în teritoriul de distribuţie al nervului periferic
•progresie în ore sau zile la deficit motor
![Page 152: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/152.jpg)
evoluţie
Neuropatia periferică
•asimetric: mononevrita multiplex •simetric: polineuropatie senzitivo-motorie
![Page 153: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/153.jpg)
rar
Neuropatia periferică
•debut insidios cu neuropatie distală senzitivă •afectarea plexului brahial
![Page 154: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/154.jpg)
rar
Afectarea SNC
•convulsii
•stroke hemoragic
![Page 155: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/155.jpg)
Afectarea renală - 70% cazuri
•proteinurie, rar sindrom nefrotic
•anomalii sediment urinar
•25% HTA secundară
•infarct renal unic sau multiplu
•IR
![Page 156: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/156.jpg)
Afectarea gastro-intestinală - 70% cazuri
•durere abdominală ce nu corelează cu viscerul afectat
•tromboză mezenterică
•hematemeză şi melenă
![Page 157: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/157.jpg)
Afectarea cardiacă - 70% cazuri
•IMA silenţios
•ICC secundară HTA sau afectării coronare
![Page 158: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/158.jpg)
Laborator
![Page 159: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/159.jpg)
•sindrom inflamator
•eozinofilie, îndeosebi în co-interesările pulmonare
•trombocitoza
•hipergamaglobulinemie
•FR
•pANCA
![Page 160: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/160.jpg)
•C3 şi C4 scăzute în boala activă în 25% cazuri
•CI crescute
•atg. şi atc. antiHBs în 10-54% cazuri
•atc. antivirus C 5% cazuri
•CRG prezente
![Page 161: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/161.jpg)
Diagnostic
![Page 162: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/162.jpg)
Biopsia •nerv sural ghidat EMG
•cutanată
•renală
•musculară (aa. musculare)
•testiculară
![Page 163: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/163.jpg)
Arteriografia •adiţională biopsiei
•reflectă extensia bolii = prognostic •diagnosticul afectării viscerelor ce nu sunt accesibile biopsiei
![Page 164: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/164.jpg)
Tratament
![Page 165: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/165.jpg)
Corticoterapie •40-60 mg/zi pentru afectare moderată
•scădere lentă după obţinere răspuns
•60-100 mg/zi sau puls-terapie pentru afectare severă progresivă
![Page 166: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/166.jpg)
Imunosupresoare •ineficienţă/toxicitate corticoterapie
•afectare severă progresivă
Ciclofosfamidă Clorambucil
Azatioprina
![Page 167: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/167.jpg)
Alte terapii
•Gamaglobuline iv
•Interferon alfa pentru PAN asociată cu virus B sau C
![Page 168: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/168.jpg)
Prognostic
![Page 169: vasculite 2014](https://reader035.vdocuments.pub/reader035/viewer/2022081422/5695d0b51a28ab9b02938ba6/html5/thumbnails/169.jpg)
Supravieţuire •80% la 7 ani cu tratament
•recidive în 40% cazuri
• majoritatea în I-ul an prin vasculită necontrolată sau infecţii •ulterior prin complicaţiile tratamentului sau deces CV
Deces