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BOALA MIXTBOALA MIXTDEDE
ESUTESUT
CONJUNCTIVCONJUNCTIV
-- Sindrom SHARPSindrom SHARP --
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DEFINDEFINIIEIIE
Combinaie de aspecte ale :
- lupus eritematos sistemic
- scleroz sistemic- polimiozit
? poliartrit reumatoid
Anticorpi anti-nRNP Descris n 1972 ce ctre SHARP
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COLAGENOZE MIXTECOLAGENOZE MIXTE-- sindrom overlapsindrom overlap --
Criterii pentru o boal(ex. lupus eritematos sistemic)
Elemente specifice altei boli(ex. factor reumatoid + leziuni
erozive) = rupus ! Aspect prezent la debut
Aspect ce apare dup debutul uneianumite boli (n 25% din colagenoze)
Sindromul Sjogren !!- asociere
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INCIDENA BMTCINCIDENA BMTC
Femei de 15 ori mai mult !
Vrsta 30-40 ani (limite 5-80 ani)
Frecvena :
LES - BMTC - SS/PM
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ASPECT CLINICASPECT CLINICDEBUTDEBUT
de obicei insidios artralgii
fenomen Raynaud, edem mnrar febr
foarte rar acut febril
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EDEM AL MINIIEDEM AL MINIIDEGETE N CRNCIORDEGETE N CRNCIOR
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Mini n BMTC : DM + SSMini n BMTC : DM + SSDMDM
--eritem Gottron,eritem Gottron,
SSSS -- sclerodactilie, degete fixate n flexiesclerodactilie, degete fixate n flexie
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clinic BMTC LES SS PM
Raynaud +++ + +++ +
Esofag +++ +++
FID +++ + +++Artrita +++ +++ +
Miozita +++ + + +++
Sclerozadifuz
+++
Renal + +++ +++
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PARA-
CLINIC
BMTC LES SS PM
Leuco-penie
++ +++ 0 0
Anti-U1RNP +++ + 0 0
Anti-ADNds
+ +++ 0 0
Anti-Sm !! +++ 0 0
Anti-Jo1 0 0 0 +++
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PROGNOSTICPROGNOSTIC n general mai bun dact al
bolilor din care este alctuitsindromul
Leziunea pulmonar (HTP)confer cel mai sever prognostic
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TRATAMENTTRATAMENT Antiinflamatorii In funcie de manifestarea
principal antimalarice- corticoterapie- ciclofosfamida
Rspuns bun la corticoterapie Evitm sulfasalazina, dapsona
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