SAFL- sindromul

antifosfolipidic

Definitie
Sindromul antifosfolipidic (SAFL) este o

entitate clinic i de laborator caracterizat

prin asocierea de tromboze arteriale i/sau
venoase, patologie obstetrical (avnd ca
mecanism principal trombozele n circulaia
placentar) i prezena unor markeri serologici
specifici - anticorpii antifosfolipidici (aFL)(1,2).
Frecvent SAFL poate coexista cu o alt boal
autoimun, cum este LES - SAFL secundar sau poate fi diagnosticat ca SAFL primar, fr
o boal asociat(3).

Istoric
I.- 1906-1952 Wasserman diagnosticul

luesului
reagina = primul anticorp aFL
lues a. antitreponema RHA
- a. non-antitreponema aCL
cardiolipina primul antigen FL reaciile fals
pozitive pentru lues LES!!!

Istoric
II.- 1952-1983 LA (anticoagulantul lupic)
Conley i Hartmann
Anticorp asociat cu tromboze
Descris la bolnave cu LES
III.- 1983 conceptul SAFL de GRAHAM HUGHES
- Lupus Unit/ Saint Thomas' Hospital
London/London Bridge Hospital

Prevalente generale
ANTICORPII aFL LA POPULAIA GENERAL
anticorpii aFL aCL (1-5%)
- LA (0-4%)
- anti-2-GP I (sub 2%)
St. FRAMINGHAM (peste 5000 indivizi)
- aCL 10% sub 40 ani
- 40% peste 80 ani
- 12-28% sub 6 ani

ETIOLOGIE - necunoscut
Prezena anticorpilor aFL/sindromul AFL se pot
asocia cu factori ce ar putea fi trigger pentru
SAF:
- boli colagen-vasaculare LES (50%)
- sindrom SJOGREN (40%)
- infecii sifilis, VHC. HIV, septicemie
- medicamente propranolol, fenitoin,
amoxicilin, IFN, anti-TNF
- estrogenii
- fumatul

Aspecte patogenetice
aFL trofoblast => neutrofile crescute
aFL TLR8 => TNF-alfa monocitar
TNF-alfa este crescut n SAFL primar
TNF-alfa este crescut n avortul spontan

experimental
citokinele Th2 => IL-6 i IL-4

Tromboza recenta vas

mic

Teritoriul vascular reprezint sediul principal al

procesului lezional din SAFL

Nosologic considerata o VASCULOPATIE

-edem endotelial (activare endotelial)
-lumen redus prin proliferare miointimal
-ngroarea, hialinizarea mediei
-lamina interna intact
-fibrin,trombocite (n peretele vascular
lumen )

ASPECTUL CLINIC
Aspectul poate fi acut, dar i al unei boli cu tendin la cronicizare i
deteriorare funcional progresiv
Tromboza venoas cea mai frecvent :
- flebit profund m. Inf. + embolie pulmonar
Tromboza arterial cea mai frecvent :
- tromboza cerebral AVC, AIT
MANIFESTRI CARDIACE angina, infarct, trombi intracardiaci,

leziuni valvulare, vegetaii (Libman-Sacks)

MANIFESTRI RENALE tromboze arter, ven, glomerulare
MANIFESTRI DIGESTIVE ischemie esofag, mezenter,
pancreatit acut, sindrom Budd-Chiari, infarct splenic
MANIFESTRI CUTANATE necroze, livedo reticularis, gangrene
Manifestari pulmonare

SAFL in vasculopatia SN
n SAFL manifestrile neurologice tipice- AVC i
AIT
manifestri atipice: disfuncie cognitiv,
demen, coree, cefalee, convulsii, leziuni
demielinizante (asemntoare sclerozei
multiple), mielit transvers, manifestri
psihiatrice diverse.
.

Imagine frontal de arteriografie a

carotidei interne, cu ocluzia

complet
a arterei cerebrale medii
drepte

sindrom Sneddon
este cunoscut triada:
boal cerebro-vascular (encefalit ischemic
acut non-inflamatoare),
livedo reticularis,
hipertensiune arterial,
considerat ca o form a SAFL, care evolueaz
cu AAFL n 40-60% din cazuri

Tratament
Tromboflebita se trateaz iniial cu anticoagulante

injectabile, apoi orale, int INR = 2-3. n cazul

recurenelor dozajul anticoagulantelor se
adapteaz pentru INR = 3-4 i se adaug aspirin.
Trombozele arteriale impun inta INR > 3 i
tratamentul va trebui continuat toat viaa.
n caz de AVC atitudinea este similar, dar este
nevoie n plus de profilaxie cu aspirin pentru
restul vieii.
Trombocitopenia sever se trateaz cu corticoizi,
gamaglobuline intravenos i, la nevoie, rituximab.

Tratament
un studiu cu 104 subieci triplu pozitivi pentru

anticoagulantul lupic, anticorpii anticardiolipinici i

anti-2GPI, care au fost urmrii timp de patrucinci ani; dintre acetia, la 25 de pacieni au aprut
evenimente tromboembolice (Pengo i colab.,
2011).
Aspirina nu influeneaz semnificativ incidena
tromboembolismului, potrivit unui trial randomizat
pe 98 pacieni cu anticorpi antifosfolipidici, ns
fr manifestri clinice, care au primit fie aspirin,
fie placebo (Erkan i colab., 2007)

PROFILAXIA - aFL prezeni

purttori aFL, asimptomatici /aspirina 80mg
control strict al factorilor de risc vascular

crescut tradiionali
anticoncepionale evitate
LES + LA/aCL HCC aspirin 80mg (risc
tromboze 3-4%/an)
SAFL exclusiv obstetrical/avort repetat
-/aspirina 80mg (risc tromboze 3-7%/an)

Bibliografie
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