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Purpura Henoch -Purpura Henoch -
SchonleinSchonleinProf. Dr. M. VoiculescuProf. Dr. M. Voiculescu
Centrul de Medicina Interna Nefrologie FundeniCentrul de Medicina Interna Nefrologie Fundeni
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DefinitieDefinitie- vasculita a vaselor mici care afecteaza:- vasculita a vaselor mici care afecteaza:
- pieleapielea- articulatiilearticulatiile- tubul digestivtubul digestiv- rinichiirinichii
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EpidemiologieEp
idemiologie- mai frecventa la copil fat de adultmai frecventa la copil fat de adult- varsta: 4 - 7 ani incidenta maximavarsta: 4 - 7 ani incidenta maxima- usoara predominenta masculinausoara predominenta masculina- incidenta sezon:incidenta sezon: iarna si primavaraiarna si primavara- manifestari extracutanate:manifestari extracutanate:
-articulare: 75%;articulare: 75%;- digestive: 50%;digestive: 50%;- afectare renala 20-90%afectare renala 20-90% IRCIRC
dializadializa transplanttransplant- mortalitate la copil < 1% (prin IRA)mortalitate la copil < 1% (prin IRA)
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Antigene implicate in declansareaAntigene implicate in declansareaepisoduluiepisodului
de purpura Henoch Schonlein (1)de purpura Henoch Schonlein (1)
InfectiiInfectii- infectii tract respirator sup.infectii tract respirator sup.- pojarpojar-
rubeolarubeola- varicelavaricela- paravovirus uman B19paravovirus uman B19- adenovirusuriadenovirusuri- virus coxsackievirus coxsackie- mycoplasma pneumoniaemycoplasma pneumoniae
- toxoplasma gonditoxoplasma gondi
Purpura Henoch -Schonlein
- salmonella hirschfeldiisalmonella hirschfeldii- clostridium difficileclostridium difficile- morganella morganiimorganella morganii- streptococcus pneumoniaestreptococcus pneumoniae- tuberculozatuberculoza- legionella longbeachlegionella longbeach- helicobacter pylorihelicobacter pylori
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Antigene implicate in declansareaAntigene implicate in declansareaepisoduluiepisodului
de purpura Henoch Schonlein (2)de purpura Henoch Schonlein (2)
MedicamenteMedicamente- vancomicynvancomicyn- ranitidinaranitidina-
streptokinazastreptokinaza- cefuroximacefuroxima- diclofenacdiclofenac- enalaprilenalapril- captoprilcaptopril
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Antigene implicate in declansareaAntigene implicate in declansareaepisoduluiepisodului
de purpura Henoch Schonlein (3)de purpura Henoch Schonlein (3)
DiverseDiverse- leucemii- leucemii- cancerul de sancancerul de san-
cancer pulmonar cu celule micicancer pulmonar cu celule mici- sindrom mielodisplazicsindrom mielodisplazic- boala granulomatoasa cronica autosomal recesivaboala granulomatoasa cronica autosomal recesiva- expunere la frigexpunere la frig- hipersensibilizare la antigene alimentarehipersensibilizare la antigene alimentare
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Anatomie patologicaAnatomie patologica
vasculita leucocitoclastica a vaselor micivasculita leucocitoclastica a vaselor mici
- intestinintestin hemoragii submucoase, subseroasehemoragii submucoase, subseroase- rinichirinichi glomerulonefrite focale sau difuzeglomerulonefrite focale sau difuze
caracteristic:caracteristic:
- depozite complexe imune cu IgA in vasele afectate,depozite complexe imune cu IgA in vasele afectate,mesangiumesangiuglomerular, pieleglomerular, piele
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Anatomie patologicaAnatomie patologica
in vasele afectate se gasescin vasele afectate se gasesc
- IgAIgA- C3C3- properdinaproperdina
infiltrat polimorfonuclear in peretii vasculariinfiltrat polimorfonuclear in peretii vasculari
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Microscopie opticaMicroscopie optica
- prin definitie sunt afectati numai unii dintre- prin definitie sunt afectati numai unii dintreglomeruliglomeruli(
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Microscopie opticaMicroscopie optica
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Microscopie opticaMicroscopie optica
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Microscopie opticaMicroscopie optica
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Microscopie opticaMicroscopie optica
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Microscopie opticaMicroscopie optica
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Microscopie opticaMicroscopie optica
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ImunofluorescentaImunofluorescenta
- IgA, IgG, IgM- IgA, IgG, IgM
- C3- C3- fibrina- fibrina
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ImunofluorescentaImunofluorescenta
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ImunofluorescentaImunofluorescenta
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Microscopie electronicaMicroscopie electronica
- depozite mezangiale mici subendotelial- depozite mezangiale mici subendotelial
- fibrina in capilare si spatiul Bowman in- fibrina in capilare si spatiul Bowman informele cuformele cu
necroza si excrescentenecroza si excrescente
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Microscopie electronicaMicroscopie electronica
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Microscopie electronicaMicroscopie electronica
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Microscopie electronicaMicroscopie electronica
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Microscopie electronicaMicroscopie electronica
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Clasificarea histologica a afectariiClasificarea histologica a afectariirenale (I)renale (I)
In functie de gradul hipercelularitatiimesangiale
-- Grad IGrad I
- GN cu leziuni glomerulare minimale (2%)GN cu leziuni glomerulare minimale (2%)
-- Grad IIGrad II- GN cu mezangio-proliferativaGN cu mezangio-proliferativa- GN mezangiopatia (10-32%)GN mezangiopatia (10-32%)
- crestere usoara a celularitatii mezangiale cu/faracrestere usoara a celularitatii mezangiale cu/faracateva leucocitecateva leucocitecirculantecirculante
- fara excrescente semilunare- fara excrescente semilunare
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Clasificarea histologica a afectariiClasificarea histologica a afectariirenale (II)renale (II)
In functie de gradul hipercelularitatiimesangiale
-- Grad IIIGrad III
- GN focal segmentala sau proliferativa endocapilaraGN focal segmentala sau proliferativa endocapilarafocalfocalsegmentala (20-45%)segmentala (20-45%)
- GN mezangiopatica (10-32%)- GN mezangiopatica (10-32%)- crestere usoara a celularitatii mezangiale cu/faracateva leucocite
circulante- fara excrescente semilunare- hipercelularitate mezangiala focala si segmentalamoderata-
frecvente leucocite intraluminare- depozite parietale capilare fuchsinofilice discontinue
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Clasificarea histologica a afectariiClasificarea histologica a afectariirenale (III)renale (III)
-- Grad IVGrad IV
- GN focal difuza proliferativa / proliferareGN focal difuza proliferativa / proliferareendocapilara (15%)endocapilara (15%)- proliferare mezangiala extensiva- prezenta variabila a leucocitelor intraluminare- excrescente semilunare pana la 50%
-- Grad VGrad V- proliferare difuza severaproliferare difuza severa- semilune in peste 50% din glomerulisemilune in peste 50% din glomeruli
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Clasificarea grupului international deClasificarea grupului international destudii alstudii al
bolilor renalebolilor renale
(criteriul-prezenta excrescentelor semilunare)
-- Grad IGrad I
- leziuni minimaleleziuni minimale
-- Grad IIGrad II- prolifearare mesangiala puraprolifearare mesangiala pura
-- Grad III - VGrad III - V
- proliferare mezangiala focal segmentala sau difuzaproliferare mezangiala focal segmentala sau difuzacucusemilune: 75% (gr.V)semilune: 75% (gr.V)
-- Grad VIGrad VI
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PatogeniePatogenie
complexe imune cu IgAcomplexe imune cu IgA
activeaza complementul (pe calea aleterna)activeaza complementul (pe calea aleterna)
leziuni tisulareleziuni tisulare
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h
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Diagnostic clinicDiagnostic clinic
-- DebutDebut: brusc, precedat de infectie tract: brusc, precedat de infectie tractrespirator suprespirator sup
-- Manifestari cliniceManifestari clinice: diferite in functie de: diferite in functie devarsta:varsta:
- copil:copil:- piele 98%piele 98%- intestin 72%intestin 72%
- articulatii 67%articulatii 67%- adult:adult:
- piele 93%piele 93%- intestin 50%intestin 50%- articulatii 27%articulatii 27%
Purpura Henoch -Schonlein
P H h
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Purpura Henoch -Schonlein
Diagnostic clinicDiagnostic clinic
- A- Afectare cutanata:fectare cutanata:
- leziuni purpurice sau urticarieleziuni purpurice sau urticarie
- leziuni maculopapulareleziuni maculopapulare
-pigmentarepigmentare
- leziuni confluenteleziuni confluente
-
rezolutie in aprox. 2 sapt. fara cicatricirezolutie in aprox. 2 sapt. fara cicatrici
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LocalizareLocalizare
- La copil:- La copil:
- membre inferioaremembre inferioare- fesefese
- La adult:- La adult:
- atipicaatipica
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Purpura Henoch - SchonleinPurpura Henoch - Schonlein
Prof. Dr. M. VoiculescuProf. Dr. M. Voiculescu
P H h
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Purpura Henoch -Schonlein
LocalizareLocalizare
- Manifestari articulare:- Manifestari articulare:
- articulare fara sinovitaarticulare fara sinovita- caracter migratorcaracter migrator- mai ales articulatii;e mari ale membrelor inf.mai ales articulatii;e mari ale membrelor inf.
- Manifestari gastrointestinale:- Manifestari gastrointestinale:
- dureri abdominale colicativedureri abdominale colicative- greturigreturi- varsaturivarsaturi- diaree sanghinolentadiaree sanghinolenta
sangerari masive,sangerari masive,
perforatii intenstinale,perforatii intenstinale,
invaginatiiinvaginatii50%50%
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LocalizareLocalizare
- Manifestari renale:- Manifestari renale:
- glomerulonefrite focale, difuzeglomerulonefrite focale, difuze- rar evolutie spre IRCrar evolutie spre IRC
- Clinic:- Clinic:
- hematurie: micro sau macroscopicahematurie: micro sau macroscopica- proteinurie: s. nefroticproteinurie: s. nefrotic- lipseste prin definitie HTAlipseste prin definitie HTA
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Purpura Henoch -Schonlein
Criterii de diagnosticCriterii de diagnostic
1. Purpura palpabila:1. Purpura palpabila:- leziuni cutanate hemoragice nelegate deleziuni cutanate hemoragice nelegate detrombocitopenietrombocitopenie
2. Varsta sub 20 ani la debutul bolii:2. Varsta sub 20 ani la debutul bolii:
3. Angor intestinal:3. Angor intestinal:
- dureri abdominale difuze, agravate dupa mese, saudureri abdominale difuze, agravate dupa mese, sauischemieischemie
intestinala, de obicei cu diaree sanghinolentaintestinala, de obicei cu diaree sanghinolenta
4. Infiltrat granulocitar in peretii vasculari:4. Infiltrat granulocitar in peretii vasculari:
-
biopsie cu granulocite in peretii arteriolelor saubiopsie cu granulocite in peretii arteriolelor sauvenulelorvenulelor
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Criterii de diagnosticCriterii de diagnostic
Dg. Pozitiv: cel putin 2 criterii: cel putin 2 criteriidin 4:din 4:
- sensibilitate: 87,1%- sensibilitate: 87,1%
- specificitate: 87,1%- specificitate: 87,1%
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TratamentTratament
SuportivSuportiv
- simptomatic: artralgii AINSsimptomatic: artralgii AINS- chirurgical: complicatiile gastrointestinalechirurgical: complicatiile gastrointestinale
Afectare renala cu degradarea functiei renale:Afectare renala cu degradarea functiei renale:
- Corticosteroizi:Corticosteroizi:
- doza atac: 0,5 mg / kg / zi, oral, 14 21 zile- doza atac: 0,5 mg / kg / zi, oral, 14 21 zile- doza intretinere10 mg / zi, oral, zilnic, 3 6 luni- doza intretinere10 mg / zi, oral, zilnic, 3 6 luni
- Ciclofosfamide:Ciclofosfamide: numai pentru cazurile cu evolutienumai pentru cazurile cu evolutiegravagrava
- 1,5 2 mg / kg / zi, zilnic, 3 6 luni- 1,5 2 mg / kg / zi, zilnic, 3 6 luni
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PrognosticPrognostic
- remisiune spontana dupa unul sau mai multe- remisiune spontana dupa unul sau mai multerecurente.recurente.
- evolutie buna chiar la cazuri netratate- evolutie buna chiar la cazuri netratate
Afectare renalaAfectare renala
- mortalitate 1% la copiimortalitate 1% la copii
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