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LESLES BoalaBoala dede etiologieetiologie necunoscutanecunoscuta caracterizatacaracterizata printrprintr--unun
LESLES
BoalaBoala dede etiologieetiologie necunoscutanecunoscuta caracterizatacaracterizata
printrprintr--unun procesproces inflamatorinflamator difuzdifuz –– afecteazaafecteaza maimai
multemulte tesuturi,tesuturi, organeorgane,, sisteme.sisteme.
--AsociazaAsociaza productiaproductia uneiunei gamagama variatevariate dede autoauto--AcAc
implicatiimplicati inin distrugeridistrugeri celularecelulare dede tiptip R.I.R.I. –– citoliticacitolitica
sausau formareformare dede CICI
-- PolimorfismPolimorfism clinicclinic sisi biologic,biologic, evolutieevolutie cucu
exacerbariexacerbari sisi remisiuneremisiune
•• IncidentaIncidenta :: »» ApareApare lala oiceoice varstavarsta,, max.max. 1515--4545 aniani ↓↓ subsub 1010
•• IncidentaIncidenta ::
»» ApareApare lala oiceoice varstavarsta,, max.max. 1515--4545 aniani
↓↓ subsub 1010 aniani sisi pestepeste 50ani50ani
»» F/BF/B == 55--9/19/1 perioadaperioada genitalagenitala activaactiva
3/13/1 tineri/tineri/varstnicivarstnici
»» PrevalentaPrevalenta:: 1515--50/100000/an50/100000/an
EtiologiaEtiologia –– necunoscutanecunoscuta == FactoriFactori declansatori/favorizantideclansatori/favorizanti
EtiologiaEtiologia –– necunoscutanecunoscuta ==
FactoriFactori declansatori/favorizantideclansatori/favorizanti
••
1.1. F.F. geneticigenetici::
»» A)A) AgregareaAgregarea familialafamiliala :: rudelerudele dede gradulgradul II
== 66--8x8x maimai frecventfrecvent LESLES frecventafrecventa crescutacrescuta
aa bolilorbolilor autolimuneautolimune inin acesteaceste familiifamilii
»» B)B) gemeniigemenii monozigotimonozigoti :: 50%50% concordantaconcordanta
LESLES
»» C)C) ↑incidentei↑incidentei unorunor markerimarkeri geneticigenetici ::
HLAHLA –– A1,A1, B8,B8, DR,DR, DQDQ
-- DR2DR2 == R.I.R.I. exageratexagerat lala AgAg nuclearenucleare
-- DR3DR3 == induceinduce hiperactivitatehiperactivitate
imuna,imuna, generalizatageneralizata
•• D)D) DeficienteDeficiente geneticegenetice aleale C2C2 sisi C4C4 :: --
•• D)D) DeficienteDeficiente geneticegenetice aleale C2C2 sisi C4C4 ::
-- ↓neutralizarea/clearanceul↓neutralizarea/clearanceul
AgAg nonnon--selfself
-- genegene vecinevecine cucu HLAHLA –– DrDr 3B83B8
•• E)E) DeficientaDeficienta geneticagenetica inin acetilareaacetilarea
hepaticahepatica aa medicamentelormedicamentelor ::
-- acetilatoriacetilatori lentilenti inin LESLES
-- deficientadeficienta inin exprimareexprimare TNFTNF
alfaalfa
2.2. F.F. hormonalihormonali : •• PreponderentaPreponderenta femeninafemenina// per.per. ActivaActiva genitalgenital
2.2. F.F. hormonalihormonali :
•• PreponderentaPreponderenta femeninafemenina// per.per. ActivaActiva genitalgenital
•• ↑↑ C%C% metabolitilormetabolitilor estrogeniestrogeni
•• ↑↑ C%C% dede prolactinaprolactina
•• AgrevareaAgrevarea LESLES –– contraceptivecontraceptive orale/sarcinaorale/sarcina
RolulRolul imunomodulartorimunomodulartor alal hormonilorhormonilor :: ↑↑
estrogeniestrogeni // ↓↓ androgeniandrogeni
HormoniiHormonii estrogeniestrogeni ::
-- ↓↓ LTsCD8+LTsCD8+
-- ↓↓ capacitateacapacitatea dede clearanceclearance alal CICI alal
McfMcf
-- ↑↑ productiaproductia dede AcAc anti-anti-ADNADN
3.3. F.F. dede mediumediu :: •• a)a) RazeleRazele UVUV :: »» modificareamodificarea ANDAND
3.3. F.F. dede mediumediu ::
•• a)a) RazeleRazele UVUV ::
»» modificareamodificarea ANDAND keratinoticekeratinotice
epidermepiderm
»» ↑↑ productiaproductia dede ILIL--11 dindin
keratinocitecukeratinocitecu stimularestimulare LTLT sisi LBLB ==
AcAc antianti--ANDAND
»» DeclanseazaDeclanseaza puseulpuseul dede LESLES
•• bb )) InfectiileInfectiile viralevirale :: »» C%C% II AcAc antianti--rujeola,rujeola, rubeola,rubeola, EB,EB,
•• bb )) InfectiileInfectiile viralevirale ::
»» C%C% II AcAc antianti--rujeola,rujeola, rubeola,rubeola, EB,EB,
parainfluentaparainfluenta
»» R.XR.X aleale AcAc dindin LESLES cucu structuristructuri
viralevirale (retrovirusuri)(retrovirusuri) –– mi?mi? imetismimetism
molecularmolecular
»» ModificareaModificarea structurilorstructurilor celularecelulare ==
AcAc
»» ↑↑ expresieexpresie MHCMHC--IIII pepe cel.cel. AccesoriiAccesorii
»» ↑↑ activitateaactivitatea LTLT
»» StructuriStructuri viralevirale inin L/epiteliuL/epiteliu
glomerularglomerular
•• c)c) SuperantigeneSuperantigene microbienemicrobiene :: enterotoxineenterotoxine ++ stimulareastimularea LT,LT,
•• c)c) SuperantigeneSuperantigene microbienemicrobiene :: enterotoxineenterotoxine ++
stimulareastimularea LT,LT, LBLB policlonalapoliclonala
•• d)d) MedicamenteMedicamente ::
»» InducInduc :: hidralazina,hidralazina, procainamida,procainamida,
HINHIN
»» AgraveazaAgraveaza :: sarurisaruri Au,Au,
anticonceptionaleanticonceptionale
•• e)e) DeficienteDeficiente inin mecanismulmecanismul apoptozeiapoptozei ↑↑ II
eliminareaeliminarea LTLT
PatogenezaPatogeneza •• AutoimunitateAutoimunitate pepe terenteren modificatmodificat
PatogenezaPatogeneza
••
AutoimunitateAutoimunitate pepe terenteren modificatmodificat genetic/dobanditgenetic/dobandit
••
A.A. AnomaliiAnomalii imuneimune
–– 1.1. HiperreactivitateaHiperreactivitatea LBLB ::
»» PePe toatatoata duratadurata boliibolii (( activa/remisiune)activa/remisiune)
»» PrinPrin stimularestimulare policlonalapoliclonala (( cantitatecantitate ↑↑ dede
AAgg )) sisi ssppecificaecifica (( ↓↓ LTsLTs sisi ↑↑ LThLTh ))
–– 2.2. DeprimareDeprimare LTsLTs ::
»» PrepoderentPrepoderent per.per. ActivaActiva LESLES
»» DeficitDeficit castigatcastigat == AcAc antianti-- limfocitelimfocite
»» ConduceConduce lala ↑↑ LThCD4+LThCD4+ == ↑↑ productieproductie dede
AcAc
»» ↓↓ NKNK
•• AutoAuto AgAg :: »» ActivareaActivarea policlonalapoliclonala LBLB »» RaportRaport LThLTh/LTs/LTs
••
AutoAuto AgAg ::
»» ActivareaActivarea policlonalapoliclonala LBLB
»» RaportRaport LThLTh/LTs/LTs alteratalterat
»» MimetismMimetism molecularmolecular (R(R X)X)
»» ModificareaModificarea selfuluiselfului (( ANDAND expusexpus lala
UVUV reactioneazareactioneaza cucu AcAc anti-anti-ANDAND ))
»» DeficienteDeficiente aleale sistemuluisistemului idiotip-idiotip-
antiidiotipantiidiotip == secretiesecretie necontrolatanecontrolata dede
ACAC
•• AutoAuto AcAc antianti -- :: –– AgAg specificespecifice :: »» PePe SS 22 celulelorcelulelor
••
AutoAuto AcAc antianti -- ::
–– AgAg specificespecifice ::
»» PePe SS 22 celulelorcelulelor (( L,L, H,H, TrTr ))
»» DinDin structurastructura m.m. cel.cel.
–– AAg.g. NNespecespecifiificece ::
»» NucleareNucleare (( 95%95% ))
»» CitoplasmaticeCitoplasmatice
»» CirculanteCirculante
•• AcAc antianti--nuclearinucleari :: »» ADNdsADNds (( nativnativ )) »» ADNssADNss »» ADNdsADNds ++
•• AcAc antianti--nuclearinucleari ::
»» ADNdsADNds (( nativnativ ))
»» ADNssADNss
»» ADNdsADNds ++ ADNssADNss
»» ARNARN
»» Nucleoproteine,Nucleoproteine, histone,histone,
glicoproteineglicoproteine nuclearenucleare solubilesolubile
•• AcAc antianti –– ANDAND == ADNADN slabslab AGAG aparapar caca urmareurmare aa RXRX cucu
•• AcAc antianti –– ANDAND == ADNADN slabslab AGAG
aparapar caca urmareurmare aa RXRX cucu
altealte AgAg sisi prinprin activareactivare
policlonalapoliclonala aa LBLB
•• FormeForme ::
»» AntiAnti –– ADNdsADNds == 60%60% -- 80%,80%,
specspecifiificcitatitatee marmaree LESLES
corelatiecorelatie cucu activitateaactivitatea
bolii/lez.bolii/lez. RenalaRenala
»» ADNssADNss == aparitieaparitie sisi inin altealte boliboli
»» ADNdsADNds ++ ssss == C%C% maremare inin Les,Les, ↓↓ inin
altealte boliboli
•• B)B) AcAc antihistoneantihistone :: celcel maimai frecventfrecvent inin LESLES •• C)C) AcAc
•• B)B) AcAc antihistoneantihistone :: celcel maimai frecventfrecvent inin LESLES
•• C)C) AcAc anti-anti-glicoproteineglicoproteine nuclearenucleare solubilesolubile ::
–– AntiAnti--SMSM == specificitatespecificitate II LESLES,, 30%30% --
markermarker
–– AntiAnti--RoRo == 30%30% -40%-40% LESLES ++ SjSjögren,ögren,
LESLES neonatalneonatal severitateaseveritatea LES,LES, lala ½½ dindin
ceicei cucu C3↓C3↓
–– AntiAnti--LaLa == rarrar inin LES,LES, frecventfrecvent inin
SjSjögrenögren
–– AntiAnti--RNPRNP--UlUl == BMTCBMTC
•• 2.2. AcAc antianti--citoplasmaticicitoplasmatici :: »» AntiAnti ARNARN monocatenarmonocatenar »»
•• 2.2. AcAc antianti--citoplasmaticicitoplasmatici ::
»» AntiAnti ARNARN monocatenarmonocatenar
»» AntiAnti--ARNARN transfertransfer
»» AntiAnti-- RNPRNP ribozomalaribozomala
»» AntiAnti--proteinaproteina PP ribozomalaribozomala
(( frecventfrecvent inin LESLES ++ afaf Psihica)Psihica)
–– AcAc antianti ::
»» HematiiHematii (( 10%10% -- 65%,65%, fixeazafixeaza C3,C3,
activiactivi lala cald)cald)
»» TrombociteTrombocite
»» LimfociteLimfocite (( maimai alesales antianti –– LTa),LTa),
antianti –– cel.cel. nervoasanervoasa
•• 3.3. AcAc circulanticirculanti :: »» AnticardiolipinaAnticardiolipina == falsafalsa R.R. lueslues »»
•• 3.3. AcAc circulanticirculanti ::
»» AnticardiolipinaAnticardiolipina == falsafalsa R.R. lueslues
»» AnticoagulantiAnticoagulanti circulanticirculanti (( 10%10% -- 15%15% ))
(( VIII,VIII, IX,IX, X,X, antitrombinaantitrombina III)III)
•• 4.4. AcAc antifosfolipideantifosfolipide ::
»» DinDin m.m. cel.cel.
»» Tromboze,Tromboze, avortavort spontanspontan
B.B. LeziuniLeziuni imuneimune •• A)A) R.R. citoliticacitolitica (II)(II) –– AcAc specificispecifici antianti
B.B. LeziuniLeziuni imuneimune
••
A)A) R.R. citoliticacitolitica (II)(II) –– AcAc specificispecifici antianti
hematie/L/Tr/m.b.glom./hematie/L/Tr/m.b.glom./ cel.nerv.cel.nerv.
••
B)B) R.R. prinprin CICI (III)(III)
»» CirculanteCirculante →depunere→depunere
»» InIn situsitu :: ADNADN (IgG,(IgG, IgM)/heparanIgM)/heparan--
sulfat/lamininasulfat/laminina
–– ↓↓ epurareaepurarea CICCIC ::
»» ↓↓ nr.nr. RR FcFc ﻻﻻ pentrupentru IgGIgG :: hematiihematii sisi McfMcf →→
»» ↓↓ fagocitareafagocitarea CICCIC == depuneredepunere tisularatisulara == activareactivare
CC33,, atragereatragere ++ activareactivare PMNPMN == enzimeenzime
lizozomalelizozomale == inflamatieinflamatie c.c.
–– ↓↓ nr.nr. RR FcFc ﻻﻻ == coordonatacoordonata geneticgenetic (( HLAHLA--
DRDR22/DR/DR33))
–– DepunereaDepunerea CICI == vasevase →vasculita/tesuturi→vasculita/tesuturi == af.af.
glomeruliglomeruli
AnatomieAnatomie patologicapatologica •• LeziuniLeziuni caracteristicecaracteristice == »» fibrinoidulfibrinoidul
AnatomieAnatomie patologicapatologica
•• LeziuniLeziuni caracteristicecaracteristice ==
»» fibrinoidulfibrinoidul :: materialmaterial amorfamorf
eozinofil,eozinofil, continecontine fibrina/Ig,fibrina/Ig, C3,C3,
corpicorpi hematoxilinicihematoxilinici (( resturiresturi nc),nc),
depusdepus pepe vase/seroase/f.vase/seroase/f. colagencolagen
•• 1.1. VasculitaVasculita :: v.v. mici/marimici/mari
PMNPMN ++ edemedem periartriolarperiartriolar
→infiltrat→infiltrat cucu mononc.mononc. ++ fibrinoidfibrinoid
•• 2.2. TegumentTegument :: –– A)A) Lez.Lez. SpecificeSpecifice :: »» AcuteAcute :: --
•• 2.2. TegumentTegument ::
–– A)A) Lez.Lez. SpecificeSpecifice ::
»» AcuteAcute :: -- eritematoaseeritematoase :: epidermepiderm
subtiat,subtiat, cel.cel. BazaleBazale vcuolizate,vcuolizate,
degenerescentadegenerescenta m.b.,m.b., infiltratinfiltrat
inflamatorinflamator ++ edemedem inin derm,derm, IgIg ++ C3C3 lala
jonctiuneajonctiunea derm/epidermderm/epiderm
-- buloasebuloase :: decolaredecolare subepidermicasubepidermica
»» SubacuteSubacute :: papulepapule--eritemeritem--scuamoasescuamoase
(psoriaziforme)(psoriaziforme)
»» CroniceCronice :: discoidediscoide == hiperkeratozahiperkeratoza ==
eritem,eritem, atrofieatrofie derm,derm, dopuridopuri cornoase,cornoase,
infiltratinfiltrat inflam,inflam, jonctiuneajonctiunea dd--ee
perifolicularperifolicular
–– B)B) Lez.Lez. NespecificeNespecifice :: vasculitavasculita
leucocitoclazicaleucocitoclazica
•• 3.3. SplinaSplina :: fibrozefibroze concetrinceconcetrince “in“in bulbbulb dede ceapaceapa inin juruljurul
•• 3.3. SplinaSplina :: fibrozefibroze concetrinceconcetrince “in“in bulbbulb dede ceapaceapa
inin juruljurul a.a. centralecentrale sisi 44 penicilarepenicilare
••
4.4. SNCSNC ::
••
»» Microinfarctizari/hemoragiiMicroinfarctizari/hemoragii prinprin
vasculitavasculita necrotizantanecrotizanta
»» CICCIC depusedepuse inin plexulplexul coroidcoroid
5.5. CordulCordul ::
»» Pericardita/miocarditaPericardita/miocardita interstitialainterstitiala
»» EndocarditaEndocardita Limbman/SacksLimbman/Sacks ==
vegetatiivegetatii nebasterienenebasteriene VV mm (( riscrisc
suprainf)suprainf)
»» CoronaritaCoronarita lupicalupica –– ATSATS coronianacoroniana
precoce/gravaprecoce/grava accentuataaccentuata dede
corticoterapiecorticoterapie
•• 6.6. RinichiRinichi :: NefritaNefrita lupicalupica (OMS(OMS 2004)2004) –– TipTip II :: GNGN
•• 6.6. RinichiRinichi :: NefritaNefrita lupicalupica (OMS(OMS 2004)2004)
–– TipTip II :: GNGN mezangialamezangiala minimaminima
–– TipTip IIII :: GNGN mezangialamezangiala proliferativaproliferativa
–– TipTip IIIIII :: GNGN focalafocala
(( segmentala/proliferativa)segmentala/proliferativa)
–– TipTip IVIV :: GNGN difuzadifuza
»» FaraFara lez.lez. segmentalesegmentale
»» CuCu lez.lez. necrozantenecrozante activeactive
»» CuCu lez.lez. activeactive sisi scleroasescleroase
»» CuCu lez.lez. scleroasescleroase
–– TipTip VV :: GNGN membranoasamembranoasa (( pura/asociatapura/asociata cucu
tiptip II,II, III,III, IV)IV)
–– TipTip VIVI ;; SclerozaScleroza
lomerularalomerulara avansataavansata
ClasificareaClasificarea histologicahistologica • Lez. Active Proliferari cel. Necroza fibrinoida Semiluni
ClasificareaClasificarea histologicahistologica
• Lez. Active
Proliferari cel.
Necroza fibrinoida
Semiluni subepiteliale
Infiltrate leucocitare
Trombi hialini
Lez. Cronice
Scleroza glomerulara
Semiluni fibroase
Atrofie tubulara
Fibroza interstitiala
SimptomatologieSimptomatologie •• DebutDebut :: •• »» Brusc,Brusc, zgomotos,zgomotos, manifestarimanifestari
SimptomatologieSimptomatologie
•• DebutDebut ::
••
»» Brusc,Brusc, zgomotos,zgomotos, manifestarimanifestari
plurivisceralepluriviscerale
»» Insidios,Insidios, afectareafectare monosistematicamonosistematica
cucu extindereextindere ulterioaraulterioara
FactoriFactori declansatoriideclansatorii ::
••
»» SoareSoare (UV),(UV), sarcina,sarcina, stres,stres, etc.etc.
SemneSemne generalegenerale ::
»» lala debut/perioadadebut/perioada activaactiva
»» Fatigabilitate,Fatigabilitate, astenieastenie
»» FebraFebra (dd(dd cucu PR)PR) –– raspunsraspuns promptprompt
P,P, AINSAINS
NuNu == infectieinfectie
»» ↓↓ GG
»» Inapetenta/anorexieInapetenta/anorexie
ManifestariManifestari specificespecifice :: •• I.I. M.M. ArticulareArticulare :: »» 90%90% »»
ManifestariManifestari specificespecifice ::
•• I.I. M.M. ArticulareArticulare ::
»» 90%90%
»» PoliartritaPoliartrita simetricasimetrica,, artic.artic. micimici
((subacsubac././crcr))
»» RedoareRedoare matinalamatinala maimai micamica (50%)(50%)
»» AbsentaAbsenta eroziunilor/eroziunilor/distructiilordistructiilor
osteoosteo--cartilaginoasecartilaginoase
»» TumefactiiTumefactii articularearticulare == inflamatieinflamatie
partiparti moimoi periartic.periartic. sisi capsulo-capsulo-
ligamentareligamentare
II.II. M.M. MusculareMusculare •• MiozitaMiozita lupicalupica (dgs.(dgs. EMg,EMg, biopsie,biopsie, rarrar ↑↑
II.II. M.M. MusculareMusculare
•• MiozitaMiozita lupicalupica (dgs.(dgs. EMg,EMg, biopsie,biopsie, rarrar ↑↑ CPK)CPK)
•• Mialgii,Mialgii, hipotrofiehipotrofie musculara,musculara, astenieastenie
muscularamusculara
III.III. M.M. osoase:osoase:
OsteonecrozaOsteonecroza asepticaaseptica :: capcap femural/humeral,femural/humeral,
carp,carp, MCMC
•• Vasculita/coticoterapiaVasculita/coticoterapia
•• AsimptomaticaAsimptomatica == imagisticaimagistica (Rgf,(Rgf, RMN,RMN, TC)TC)
••
IV.IV. M.M. cutaneo-cutaneo-mucoasemucoase :: •• 8080%,%, dgsdgs PrecocePrecoce •• A)A) EruptiaEruptia
IV.IV. M.M. cutaneo-cutaneo-mucoasemucoase ::
•• 8080%,%, dgsdgs PrecocePrecoce
•• A)A) EruptiaEruptia eritematoasaeritematoasa facialafaciala “in“in aripiaripi dede
fluturefluture””-- vespertilio:vespertilio:
»» Pometi+dorsulPometi+dorsul nasuluinasului farafara
afectareaafectarea santuluisantului nazonazo--labiallabial
((eritemeritem fix,fix, plat/plat/supradenivelatsupradenivelat))
»» AparitiaAparitia//exacerbareexacerbare dupadupa
expunereaexpunerea lala
soaresoare/independent/independent (LES)(LES)
cutanatcutanat acutacut==4040%)%)
•• bb)) LupusulLupusul discoiddiscoid croniccronic :: »» LocalizatLocalizat (scalp,(scalp, fata,fata,
•• bb)) LupusulLupusul discoiddiscoid croniccronic ::
»» LocalizatLocalizat (scalp,(scalp, fata,fata, pavilionpavilion
ureche,ureche, brate,brate, spatespate
»» AfectareaAfectarea cutanatacutanata asemanatoareasemanatoare
LESLES
•• EritemEritem circumscriscircumscris –– papuloscuamospapuloscuamos ––
hiperkeratozahiperkeratoza –– atrofieatrofie (hipopigment)(hipopigment)
•• Lez.Lez. CutanataCutanata farafara modificarimodificari serologiceserologice
autoimune/evolutieautoimune/evolutie LESLES
•• c)c) EruptiiEruptii urticarieneurticariene
•• d)d) FotosensibilitateFotosensibilitate :: eritemeritem zonelezonele expuseexpuse lala
soaresoare (frunte,(frunte, barbie)/vasculitabarbie)/vasculita AcAc antianti--Ro+Ro+
•• E)E) VasculitaVasculita :: »» UlcereUlcere gambieregambiere perimaleolareperimaleolare »»
••
E)E) VasculitaVasculita ::
»» UlcereUlcere gambieregambiere perimaleolareperimaleolare
»» Eritem+edemEritem+edem periunghial,periunghial, necrozenecroze
parcelareparcelare pulpapulpa degetelordegetelor
»» LivedoLivedo reticularis,reticularis, S.S. RaynaudRaynaud == AcAc antuantu--
fosfolipidicifosfolipidici
»» PrupuraPrupura palpabilapalpabila,, petesiipetesii echimozeechimoze
»» UlceratiiUlceratii mucoasamucoasa nazala/bucalanazala/bucala
(sept/palat(sept/palat moalemoale--dur)dur) nedureroasenedureroase
••
F)F) Alopecie.Alopecie.
»» ParcelaraParcelara (majoritatea,(majoritatea, lala debut),debut), parpar ruptrupt
lala distantadistanta dede emergentiemergenti
»» Difuza/permanentaDifuza/permanenta (L.(L. discoid)discoid) ––
secundarasecundara terapieiterapiei
V.V.M.M. Pleuro-Pleuro-pulmonarepulmonare: •• A)A) PleureziePleurezie :: »» Cca.Cca. 30%,30%,
V.V.M.M. Pleuro-Pleuro-pulmonarepulmonare:
•• A)A) PleureziePleurezie ::
»» Cca.Cca. 30%,30%, uni/uni/bilateralabilaterala
»» SimptomatologieSimptomatologie discretadiscreta (pleurita)(pleurita)
»» SerofibrinoasaSerofibrinoasa –– exudatexudat (anti(anti--AND,AND,
↓C3,↓C3, cel.cel. LE+,LE+, CI,CI, limfocite)limfocite)
»» RaspunsRaspuns ++ lala
corticoterapie/prognosticcorticoterapie/prognostic bunbun
(cantitate(cantitate maremare == inferctioasa)inferctioasa)
•• B)B) PneumoniaPneumonia ac.ac. carecare evolueazaevolueaza •• C)C) P.I.F.:P.I.F.: »» Difuza,Difuza,
•• B)B) PneumoniaPneumonia ac.ac. carecare evolueazaevolueaza
•• C)C) P.I.F.:P.I.F.:
»» Difuza,Difuza, frecventfrecvent asimptomaticaasimptomatica
»» Dispneee/tuseDispneee/tuse seacaseaca (sputa(sputa
hemoptoica)hemoptoica) crepitantecrepitante bazebaze
»» Functionala:Functionala: DRR,DRR, tulburaritulburari dede
difuziunedifuziune (bloc(bloc AA--C)C) == HTPHTP
»» RxRx :: fibrozafibroza bazalabazala difuzadifuza reticuloreticulo--
nodularanodulara
»» EvolutieEvolutie bunabuna subsub corticoterapiecorticoterapie
VI. M.C-V •• A)A) PericarditaPericardita :: »» 1/31/3 dindin bolnavibolnavi (poliserozita)(poliserozita) »»
VI. M.C-V
•• A)A) PericarditaPericardita ::
»» 1/31/3 dindin bolnavibolnavi (poliserozita)(poliserozita)
»» F.F. rarrar tamponada/pericarditatamponada/pericardita
constrictivaconstrictiva
»» EvolutieEvolutie bunabuna subsub corticoterapiecorticoterapie
•• B)B) MiocarditaMiocardita ::
»» TT 00 ,, tahicardie,tahicardie, TT ritm/conducereritm/conducere
»» I.I. cardiacacardiaca (HTA(HTA renovasculara)renovasculara)
»» AsociereAsociere frecventafrecventa cucu miozitamiozita sisi
serozitaserozita
• C) EndocarditaEndocardita LibmanLibman--Sacks:Sacks: »» II mm /Ao,/Ao, prolapsprolaps VV mm (leziuni(leziuni dede
• C) EndocarditaEndocardita LibmanLibman--Sacks:Sacks:
»» II mm /Ao,/Ao, prolapsprolaps VV mm (leziuni(leziuni dede
cordaj)cordaj)
»» TT 00 ,, sulfusulfu sistolic,sistolic, anemieanemie
•• D)D) CoronaritaCoronarita lupicalupica :: IMacIMac frecventfrecvent (vasculita(vasculita
++ CST)CST)
•• E)E) TromboflebiteTromboflebite superficiale/profundesuperficiale/profunde == AcAc
anticoagulantianticoagulanti inin serser preponderentpreponderent inin m.m. inf.inf.
→TEP→TEP
•• F)F) TrombozeTromboze arterialearteriale :: cerebralecerebrale sisi membremembre
•• VII.VII. M.M. digestivedigestive :: –– Greata,Greata, varsaturi,varsaturi, inapetentainapetenta ––
•• VII.VII. M.M. digestivedigestive ::
–– Greata,Greata, varsaturi,varsaturi, inapetentainapetenta
–– DureriDureri abdominale:abdominale:
»» VasculitaVasculita mezentericamezenterica –– perforatieperforatie
»» Peritonita/serozitaPeritonita/serozita +/+/-- exudatexudat
»» UGDUGD == CSTCST
»» PancreatitaPancreatita == AzatioprinaAzatioprina
–– hepatomegaliehepatomegalie :: rar/vasculitarar/vasculita –– trombozetromboze
venoasevenoase
•• VIII.VIII. SplenomegalieSplenomegalie :: relativrelativ frecventafrecventa (20%,(20%, uneoriuneori
infarctizari)infarctizari)
•• IX.IX. PoliadenopatiePoliadenopatie ::
»» frecventfrecvent generalizata/laterocervicalageneralizata/laterocervicala
(dd)(dd)
X.X. M.M. HematologiceHematologice :: •• A)A) Anemie:Anemie: »» FrecventFrecvent moderatamoderata »»
X.X. M.M. HematologiceHematologice ::
•• A)A) Anemie:Anemie:
»» FrecventFrecvent moderatamoderata
»» Normocroma/citara;Normocroma/citara; hipocromahipocroma--
microcitaramicrocitara--hiposiderremicahiposiderremica
»» HemoliticaHemolitica 50%50%
•• B)B) TrombocitopenieTrombocitopenie ::
subsub 100100 000/mm000/mm 33 /inaugurala/inaugurala (purpura,(purpura,
echimoze,echimoze, hemoragiehemoragie cerebrocerebro--meningee)meningee)
•• CC)) LeucopenieLeucopenie cucu limfocitopenielimfocitopenie
XII.XII. M.M. RenaleRenale :: –– 60%;60%; prognosticprognostic negativnegativ –– ClinicClinic ::
XII.XII. M.M. RenaleRenale ::
–– 60%;60%; prognosticprognostic negativnegativ
–– ClinicClinic :: proteinarieproteinarie persistentapersistenta (70%),(70%),
hematurie,hematurie, cilindriicilindrii edeme,edeme, HTA,HTA, retentieretentie
azotataazotata
–– PBRPBR == tipultipul dede nefropatienefropatie
–– GNGN difuzadifuza :: proteinurieproteinurie pestepeste 3,5g/24h3,5g/24h
hematurie,hematurie, cilindrurie,cilindrurie, HTAHTA renovasculara,renovasculara,
IRc,IRc, AcAc antianti--ADNds,ADNds, ↓↓ C3C3 (SN(SN impur/PBR)impur/PBR)
XII.XII. M.M. NeuroNeuro--psihicepsihice :: •• SemnSemn dede gravitategravitate •• SindromSindrom organicorganic
XII.XII. M.M. NeuroNeuro--psihicepsihice ::
•• SemnSemn dede gravitategravitate
•• SindromSindrom organicorganic cerebralcerebral :: anxietate,anxietate, insomnie,insomnie,
depresie,depresie, tulb,tulb,
memorie/orientare/perceptie/intelect/memorie/orientare/perceptie/intelect/ labilitatelabilitate
psihica/halucinatii/confuzie/excitatiepsihica/halucinatii/confuzie/excitatie
•• PsihozaPsihoza lupicalupica :: dezorientaredezorientare temporotemporo--spatialaspatiala (Ac(Ac
antianti--proteinaproteina PP ribozom)ribozom) (dd(dd cucu CST)CST)
•• CrizeCrize comitialecomitiale localizate/generalizatelocalizate/generalizate •• DeficitDeficit motormotor
•• CrizeCrize comitialecomitiale localizate/generalizatelocalizate/generalizate
•• DeficitDeficit motormotor centralcentral :: hemiplegie/hemiparezahemiplegie/hemipareza
•• MielitaMielita acutaacuta transversatransversa (rar)(rar) :: paraparezaparapareza
•• CefaleeCefalee severasevera
•• NeuropatieNeuropatie periferica/n.periferica/n. cranienecraniene
XIII.XIII. M.M. OculareOculare :: •• SpasmSpasm retinianretinian (vasculita);(vasculita); FOFO == corpicorpi
XIII.XIII. M.M. OculareOculare ::
•• SpasmSpasm retinianretinian (vasculita);(vasculita); FOFO == corpicorpi citoizicitoizi
(infarct)(infarct)
•• GlaucomGlaucom cucu unghiunghi inchis/S.inchis/S. sicasica (( S.S. SjSjögren)ögren)
XIV.XIV. LESLES sisi sarcinasarcina :: •• EfectEfect :: agravareagravare LES/declansareLES/declansare puse/puse/
XIV.XIV. LESLES sisi sarcinasarcina ::
•• EfectEfect :: agravareagravare LES/declansareLES/declansare puse/puse/
evolutieevolutie severa/frecventsevera/frecvent ecampsieecampsie
•• RiscRisc fetalfetal ::
»» avortavort spontanspontan
»» nasterenastere prematura/fatprematura/fat mortmort
•• SindromulSindromul dede lupuslupus neonatalneonatal ::
»» 50%50% dindin mamemame suntsunt asimptomaticeasimptomatice
AcAc antianti--RoRo lala mamamama == BL.BL. A-A-VV gr.gr.
IIIIII lala fatfat persistentapersistenta canalcanal arterial,arterial,
rashrash
»» +Ac+Ac antianti--LALA (5%),(5%), HLAHLA--B8,B8, DR3DR3
LaboratorLaborator •• AnemieAnemie :: »» normocroma/normocroma/-- citaracitara »»
LaboratorLaborator
•• AnemieAnemie ::
»» normocroma/normocroma/-- citaracitara
»» Hipocrom/miocrocitareHipocrom/miocrocitare
»» HemoliticaHemolitica (T.(T. Coombs+,Coombs+, AcAc anzianzi--
hematii+,hematii+, reticulocitoza,reticulocitoza, II BI)BI)
•• LeucopenieLeucopenie cucu limfocitopenielimfocitopenie (2500(2500--400)400)
•• TrombocitopenieTrombocitopenie (poate(poate precedeprecede cucu aniani LES)LES) ::
refractararefractara lala tratament/beningnatratament/beningna
•• ↑↑ VSHVSH inin puseupuseu (( N=crioglobuline+)N=crioglobuline+)
•• CPRCPR == NN sausau f.f. putinputin ↑↑ (( infectiiinfectii supraadaugate)supraadaugate)
•• ↓↓ haptoglobina,haptoglobina, ↑↑ amiloidulamiloidul AA
•• ↑↑ alfaalfa 22 inin puseu,puseu, hipergamaglobulinemiehipergamaglobulinemie
policlonalapoliclonala (75%)(75%)
•• Cel.Cel. LELE (70%(70%--80%):80%): »» PMNPMN cucu nc.nc. impinseimpinse perifericperiferic dede
•• Cel.Cel. LELE (70%(70%--80%):80%):
»» PMNPMN cucu nc.nc. impinseimpinse perifericperiferic dede
corpusucululcorpusuculul lupiclupic fagocitafagocita
»» RozetaRozeta luluppicaica
»» CorpuscululCorpusculul lupiclupic :: nc.lezatnc.lezat dede acac
antianti--nc/citoplasmatici/masanc/citoplasmatici/masa amorfaamorfa
expulzataexpulzata sisi fagocitatefagocitate dede PMNPMN
•• AutoAcAutoAc :: imunoflorescentaimunoflorescenta indirecta/raioimunologieindirecta/raioimunologie –– acac
•• AutoAcAutoAc :: imunoflorescentaimunoflorescenta
indirecta/raioimunologieindirecta/raioimunologie
–– acac antianti--nuclearinucleari (( pestepeste 95%)95%) cucu
imunofluorescentaimunofluorescenta tiptip ::
••
»» InelarInelar == ADNdsADNds
»» PatatPatat == Sm,Sm, Ro,Ro, LaLa
»» NuclearNuclear == RNPRNP nuclearenucleare
»» OmogenOmogen == antihistoneantihistone
–– AcAc antianti--citoplasmatici,citoplasmatici, anti-anti-fodfolipide,fodfolipide,
circulanticirculanti
CICCIC titrutitru ↑↑
•• CrioglobulineCrioglobuline ++ inin SS Raynaud,Raynaud, nefropatienefropatie
•• ↓↓↓↓ C3C3 inin puseupuseu ;; afectareafectare renalarenala ↓C4,↓C4, C3,C3, CH50CH50
•• FRFR falsfals ++ (( 15%15%--30%)30%)
•• Ex.Ex. urinaurina :: proteinurie/cilindrurieproteinurie/cilindrurie (celulari)/(celulari)/ hematuriehematurie
•• Ex.Ex. urinaurina :: proteinurie/cilindrurieproteinurie/cilindrurie (celulari)/(celulari)/
hematuriehematurie
•• RetentieRetentie azotataazotata inin IRen/IRen/ PBRPBR –– tiptip histologichistologic
GNGN
•• RxRx pulmonarpulmonar ++ punctiepunctie pleuralapleurala (dd(dd TBC)TBC)
•• AlteAlte exameneexamene TC,TC, EEG,EEG, RMNRMN
FormeForme cliniceclinice :: variate/fct.variate/fct. dede organulorganul afectatafectat •• A.A. LupusLupus
FormeForme cliniceclinice :: variate/fct.variate/fct. dede
organulorganul afectatafectat
•• A.A. LupusLupus iatrogeniatrogen :: pestepeste 5050
medicamente/produsemedicamente/produse alimentare/cosmeticealimentare/cosmetice
–– InducInduc :: hidralazinahidralazina (150mg/zi(150mg/zi timptimp indelungat),indelungat),
procainamida,procainamida, DFH,DFH, HIN,HIN, DD--penicilamina,penicilamina,
alfaalfa--metildopametildopa
–– AgraveazaAgraveaza :: penicilina,penicilina, sulfamidele,sulfamidele, sarurisaruri Au,Au,
contraceptivelecontraceptivele oraleorale
•• Particularitati:Particularitati: »» RepartitieRepartitie egalaegala pepe sexesexe »» LipsaLipsa
•• Particularitati:Particularitati:
»» RepartitieRepartitie egalaegala pepe sexesexe
»» LipsaLipsa afectariiafectarii renale/neurologicerenale/neurologice
»» AbsentaAbsenta AcAc antianti--ADNds,ADNds, C3=NC3=N
»» PrezentaPrezenta AcAc antianti--histonahistona 90%)90%)
»» DisparitiaDisparitia manifestarilormanifestarilor
clinice/paracliniceclinice/paraclinice inin 44--6s6s dede lala
sistareasistarea factoruluifactorului incriminatincriminat
•• B.B. SindromulSindromul anifosfolipidic:anifosfolipidic: •• Primar/secundarPrimar/secundar (LES)(LES) ––
•• B.B. SindromulSindromul anifosfolipidic:anifosfolipidic:
•• Primar/secundarPrimar/secundar (LES)(LES)
–– AnticoagulantulAnticoagulantul lupiclupic –– acac antifosfolipideantifosfolipide ::
interfereazainterfereaza cucu fct.fct. ProcoagulantaProcoagulanta aa
fosfolipidelorfosfolipidelor inin testeletestele dede coagularecoagulare inin vitrovitro
»» riscrisc↑↑ dede trombozetromboze venoase/arterialevenoase/arteriale
»» AvortAvort dede primprim trimestrutrimestru repetitivrepetitiv
(tromboza(tromboza dede venevene placetare)placetare)
»» AbsentaAbsenta uneiunei sangerarisangerari excesiveexcesive
»» NecesitaNecesita tratamenttratament profilacticprofilactic
anticoagulentanticoagulent lala ceicei cucu trombozetromboze
•• AcAc nonnon--neutralizantineutralizanti fatafata dede protrombinaprotrombina (grup(grup redusredus )) == ––
•• AcAc nonnon--neutralizantineutralizanti fatafata dede protrombinaprotrombina (grup(grup
redusredus )) ==
–– HipoprotrombinemieHipoprotrombinemie cucu sangerarisangerari anormaleanormale
–– PrelungireaPrelungirea PTTPTT (TP(TP == NN sausau foartefoarte putinputin
prelungit)prelungit)
–– TratamentTratament cucu CSTCST
•• AnticoagulantulAnticoagulantul lupiclupic sese leagaleaga dede protrombinaprotrombina –– AcAc
•• AnticoagulantulAnticoagulantul lupiclupic sese leagaleaga dede protrombinaprotrombina
–– AcAc antifosfolipidiciantifosfolipidici reactioneazareactioneaza cucu epitopiepitopi aiai
proteineiproteinei carecare faceface complexcomplex cucu fosfolipidelefosfolipidele
–– AcAc anticadiolipinaanticadiolipina –– sese leagaleaga dede betabeta--22
glicoproteinaglicoproteina II (( VDRLVDRL fals+)fals+)
–– AmbiiAmbii AcAc sese leagaleaga dede proteineleproteinele C,C, SS
DiagnosticulDiagnosticul ++ alal LESLES (criteriile(criteriile ACR)ACR) •• Dgs+Dgs+ == 4/114/11 –– LESLES
DiagnosticulDiagnosticul ++ alal LESLES (criteriile(criteriile
ACR)ACR)
•• Dgs+Dgs+ == 4/114/11
–– LESLES incompletincomplet (latent(latent )) :: 11--33 +/+/-- altealte manifestarimanifestari
neincluseneincluse inin criteriicriterii
EvolutieEvolutie
•• CuCu puseepusee dede acutizareacutizare sisi perioadeperioade dede remisiuneremisiune
–– FactoriFactori declansatorideclansatori :: UVUV (20%),(20%), sarcina,sarcina,
medicamente,medicamente, infectiiinfectii
ComplicatiiComplicatii • A. Alte bolii : –– 1.1. AfectareaAfectarea renalarenala :: GNGN difuzadifuza→→ ICRICR
ComplicatiiComplicatii
• A. Alte bolii :
–– 1.1. AfectareaAfectarea renalarenala :: GNGN difuzadifuza→→ ICRICR == ceacea maimai
frecventafrecventa cauzacauza dede decesdeces
–– 2.2. AfectareaAfectarea neurologicaneurologica :: vasculitavasculita cerebralacerebrala sausau
hemoragiehemoragie cerebro/meningeecerebro/meningee cucu hemiplagiehemiplagie
–– 3.3. AfectareaAfectarea cardiacacardiaca ::
»» MiocarditaMiocardita == ICIC
»» PericarditaPericardita == tamponadatamponada
»» EndocarditaEndocardita verucoasaverucoasa == infectioasa/ICinfectioasa/IC
–– 4.4. AfectareAfectare coronarianacoronariana :: IMacIMac (( ATSATS
favorizata/agravatafavorizata/agravata dede CTS/CTS/ inflamatia)inflamatia)
•• B.B. AleAle tratamentuluitratamentului :: •• 1.1. NeoplaziiNeoplazii == imunosupresoareimunosupresoare
•• B.B. AleAle tratamentuluitratamentului ::
•• 1.1. NeoplaziiNeoplazii == imunosupresoareimunosupresoare (ciclofosfamida(ciclofosfamida ==
neo/supresieneo/supresie medulara)medulara)
•• 2.2. InfectiiInfectii :: CTSCTS ++ imunosupresoareimunosupresoare
»» GermeniGermeni pigeni/Gpigeni/G--?BK/pneumosistis?BK/pneumosistis
cariniicarinii
»» Candida/virusuriCandida/virusuri
PrognosticPrognostic
AmelioratAmeliorat dede terapieterapie :: 50%50% -- pestepeste 5a5a // 90%90% pestepeste 10a10a
rezervatrezervat lala ceicei cucu nefropatienefropatie sausau afectareafectare neuroneuro--
psihicapsihica
TratamentTratament •• A.A. MasuriMasuri nefarmacologicenefarmacologice –– 1.1. ProfilaxiaProfilaxia
TratamentTratament
•• A.A. MasuriMasuri nefarmacologicenefarmacologice
–– 1.1. ProfilaxiaProfilaxia recidivelorrecidivelor –– evitareaevitarea factorilorfactorilor
dede risc:risc:
»» ExpunereaExpunerea directa/reflectatadirecta/reflectata lala
soaresoare
»» SarcinaSarcina “(“( pestepeste 3a3a dede lala ultimulultimul
puseupuseu ?)?)
»» AdministrareaAdministrarea nemotivatanemotivata dede
medicamentemedicamente (( contraceptiva)contraceptiva)
»» EforturiEforturi fizicefizice mari,mari, stresstres
–– 2.2. IngienoIngieno--dieteticdietetic ::
»» repausrepaus prelungitprelungit inin puseupuseu sisi inin afaraafara
»» DietaDieta adaptataadaptata lala afectiuniafectiuni
(nefro)/CTS(nefro)/CTS
•• B.B. TratamentTratament medicamentosmedicamentos –– 1.1. AspirinaAspirina sisi AINSAINS
•• B.B. TratamentTratament medicamentosmedicamentos
–– 1.1. AspirinaAspirina sisi AINSAINS nonaspirinicenonaspirinice ::
–– 2.2. AntimalariceAntimalarice dede sintezasinteza ::
•• InIn lupuslupus discoiddiscoid cronic/cronic/formeleformele usoareusoare dede LESLES
•• UtileUtile inin asociereasociere cucu AINSAINS
•• HidroxiclorochinaHidroxiclorochina == 400400--600mg/600mg/zizi (2-(2-3cps)3cps)
dozadoza dede atacatac XX 44--66 saptamanisaptamani
–– 3.3. CortcosterroiziiCortcosterroizii ::
•• terapieterapie dede bazabaza/de/de catrecatre oriori esteeste nevoienevoie
•• 4.4. ImunosupresoareleImunosupresoarele :: •• –– InIn formeleformele severesevere dede LESLES
•• 4.4. ImunosupresoareleImunosupresoarele ::
••
–– InIn formeleformele severesevere dede LESLES (renale,(renale, neurologie)neurologie)
–– CandCand CSTCST suntsunt ineficientiineficienti lala dozedoze marimari
(corticorezistenta)(corticorezistenta)
–– Corticodependenta/Corticodependenta/ c.i.c.i. pentrupentru CSTCST
CiclofosfamaidaCiclofosfamaida ::
•• 2,5mg/Kcorp/zi2,5mg/Kcorp/zi (50mg/cp)(50mg/cp)
•• PlusPlus terapieterapie :: 0,70,7 –lg/luna–lg/luna ivpivp XX panapana lala
7g/an)7g/an) (10(10--15mg/K/ZI)15mg/K/ZI) apoiapoi p.op.o
•• AzatiprinaAzatiprina :: 1,51,5--22 mg/K/zimg/K/zi atacatac (nefrita)(nefrita) dede
novo/dupanovo/dupa cicluciclu
•• MTXMTX :: 7,515mg/sapt7,515mg/sapt
•• MycofenolatMycofenolat mofetilmofetil
•• 5.5. PlasmaferezaPlasmafereza :: •• dede exceptieexceptie (boala(boala grava,grava, refractararefractara lala
•• 5.5. PlasmaferezaPlasmafereza ::
•• dede exceptieexceptie (boala(boala grava,grava, refractararefractara lala
tratament)tratament) cucu titrutitru ↑↑ DEDE CI,CI, AcAc anti-anti-ADNdsADNds
•• 33--4L/sapt,4L/sapt, apoiapoi plusplus terapieterapie cucu
ciclofosfamidaciclofosfamida
•• 6.6. AcAc monoclonalimonoclonali antianti--TNFTNF
•• 7.7. Hemodializa,Hemodializa, transplanttransplant renalrenal lala ceicei cucu evolutieevolutie
sprespre IRenIRen
•• 88 Danazol/imunoglobulineDanazol/imunoglobuline iviv (300-(300-400mg/K/zi400mg/K/zi xx
55 zilezile dacadaca nunu ↑↑ dupadupa Danazol)Danazol)
•• 9.9. DapsonaDapsona :: inin lez.lez. CutanateCutanate
•• 10.10. TratamentulTratamentul complicatiilorcomplicatiilor
StrategiaStrategia tratamentuluitratamentului •• AnomaliileAnomaliile bilogicebilogice inin absentaabsenta
StrategiaStrategia tratamentuluitratamentului
••
AnomaliileAnomaliile bilogicebilogice inin absentaabsenta
simptomatologieisimptomatologiei cliniceclinice NUNU sese trateazatrateaza
•• NuNu esteeste unun beneficiubeneficiu tratamentultratamentul profilacticprofilactic cucu
dozedoze micimici dede PP
•• CeiCei cucu manifestarimanifestari articulare,articulare, moderatmoderat activeactive potpot
beneficiabeneficia dede AINS,AINS, hidroxiclorochina,darhidroxiclorochina,dar nunu
justificajustifica PP
•• ImunosupresiveleImunosupresivele sese restangrestang lala ceicei cucu afectareafectare
renala/neurologicarenala/neurologica
BoalaBoala mixtamixta aa tesutuluitesutului conjuctivconjuctiv (S.(S. Sharp)Sharp) • Semne clinice de LES, SD, PM +
BoalaBoala mixtamixta aa tesutuluitesutului conjuctivconjuctiv
(S.(S. Sharp)Sharp)
• Semne clinice de LES, SD, PM + titru ↑ de AC
anti-RNP-U1
• Etiologia = necunoscuta (F/B = 9/1)
•• PatogenezaPatogeneza :: imunaimuna == fenomenefenomene autoimuneautoimune prinprin mimetismmimetism
•• PatogenezaPatogeneza :: imunaimuna == fenomenefenomene autoimuneautoimune
prinprin mimetismmimetism molecularmolecular alal unuiunui retrovirusretrovirus v.v.
RNPRNP--U1U1
•• ArgumenteArgumente ::
»» AcAc antianti--RNPRNP--U1U1 inin titru↑titru↑
»» HipergamaglobulinemieHipergamaglobulinemie policlonalapoliclonala
»» HiperactivitateHiperactivitate aa LBLB
»» DefectDefect dede LTSLTS
»» TitruTitru ↑↑ dede CIC,CIC, ↓↓ C3C3 depozitedepozite dede
IgG/MIgG/M sisi C3C3 inin pereteleperetele
vascular/m.b.vascular/m.b. Glom.Glom.
»» InfiltratInfiltrat L,L, P1P1 inin tesuturitesuturi
•• MorfologiceMorfologice :: –– A)A) vasculitavasculita –– a.a. mici/mijlociimici/mijlocii cucu
•• MorfologiceMorfologice ::
–– A)A) vasculitavasculita –– a.a. mici/mijlociimici/mijlocii cucu proliferareproliferare
intimalaintimala
–– B)B) infiltratinfiltrat inflamatorinflamator –– gl.gl. Salivare,Salivare, ficat,ficat, tubtub
digestivdigestiv
–– C)C) anomaliianomalii capilaroscopicecapilaroscopice –– dilatate/aspectdilatate/aspect
punctatpunctat
SimptomatologieSimptomatologie :: -4-4--8080 aniani (x=40a)(x=40a) •• 1.1. ManifestariManifestari frecventefrecvente
SimptomatologieSimptomatologie :: -4-4--8080 aniani
(x=40a)(x=40a)
•• 1.1. ManifestariManifestari frecventefrecvente ::
–– S.S. RaynaudRaynaud :: poatepoate precedeprecede cucu 1/ani1/ani
simptomatologiasimptomatologia
–– PoliartritaPoliartrita :: nedeformata,nedeformata, redoareredoare matinalamatinala
MCF,MCF, IFPIFP
–– SclerodactilieSclerodactilie :: degetedegete “in“in carnaciori”,carnaciori”,
tegumentetegumente ingrosate,ingrosate, edematiateedematiate
–– DisfunctieDisfunctie esofagiana,esofagiana, s.s. dede malasorbtiemalasorbtie
–– AfectareAfectare pulmonarapulmonara :: dispnee,dispnee, dureridureri
pleurale,pleurale, HTp,HTp, RR--xx--micromicro opacitatiopacitati bazebaze
–– MiopatieMiopatie inflamatorieinflamatorie :: m.m. superioare,superioare,
astenei,astenei, ICPK,ICPK, EMG+EMG+
•• 2.2. ManifestariManifestari cucu frecventafrecventa redusa:redusa: •• EruptiiEruptii cutanatecutanate
•• 2.2. ManifestariManifestari cucu frecventafrecventa redusa:redusa:
•• EruptiiEruptii cutanatecutanate malare,malare, alopecie,alopecie,
hipo/hiperpigmentarehipo/hiperpigmentare cutanatacutanata
•• Adenopatie,Adenopatie, TT 00
•• AfectareAfectare cardiacacardiaca :: pericardita,pericardita, miocarditamiocardita
PVM,PVM, IC,IC, IAoIAo
•• AfectareAfectare renalarenala :: GNGN membranoasamembranoasa
•• AfectareAfectare neurologicaneurologica :: nevralgienevralgie trigementrigemen
•• LaboratorLaborator :: –– AcAc antianti--RNPRNP--U1U1 == pestepeste 1/101/10 000000 (( inin absentaabsenta
•• LaboratorLaborator ::
–– AcAc antianti--RNPRNP--U1U1 == pestepeste 1/101/10 000000 (( inin
absentaabsenta altoraltor AcAc antianti –– NcNc specificispecifici == marcamarca
serologicaserologica ))
–– ↓↓ NL,NL, TrTr ↑↑ VSH,VSH, anemie,anemie, ↓↓ inconstantainconstanta C3C3
–– Hipergamaglobulinemie,Hipergamaglobulinemie, CIC,CIC, FR,FR, cel.cel. LELE
–– AbsentaAbsenta AcAc antianti--ADNds/SMADNds/SM
–– BiopsieBiopsie muscularamusculara :: degenerscentadegenerscenta fibrefibre
musculare,musculare, infiltratinfiltrat inflamatorinflamator sisi depozitedepozite
liniareliniare dede IgMIgM lala jonctiuneajonctiunea D/ED/E
–– EMG,EMG, CPKCPK
•• Dgs.Dgs. ++ :: –– A)A) clinicclinic :: »» EdemEdem alal mainilormainilor »» S.S. RaynaudRaynaud
••
Dgs.Dgs. ++ ::
–– A)A) clinicclinic ::
»» EdemEdem alal mainilormainilor
»» S.S. RaynaudRaynaud
»» PoliartritaPoliartrita (sinovita)(sinovita)
»» MiozitaMiozita
»» SclerodactilieSclerodactilie
–– B)B) bilogicbilogic :: antianti--RNPRNP--U1U1 pestepeste 1/100001/10000 (( abs.abs.
altialti AcAc antianti--NespecificiNespecifici
•• TratamentTratament :: asemanatorasemanator LESLES –– a)a) FormaForma usoarausoara :: AINSAINS »»
•• TratamentTratament :: asemanatorasemanator LESLES
–– a)a) FormaForma usoarausoara :: AINSAINS
»» HidroxiclorochinaHidroxiclorochina
»» VV--dilatatoaredilatatoare (S.(S. Raynaud)Raynaud)
»» PP iinn ddozaoza mmiicaca
–– b)Formab)Forma severasevera :: PP == lmg/Kcorp/zilmg/Kcorp/zi ((
miozita,miozita, af.af. +/+/-- plusplus terapieterapie visceralaviscerala
–– c)c) FormaForma gravagrava :: P+ciclofosfamidaP+ciclofosfamida (0,5-(0,5-
lg/luna,lg/luna, apoiapoi 0,3/s0,3/s p.o.p.o. totaltotal 33--6g)6g)