SINDROME ANTIFOSFOLIPIDOS

SINDROME ANTIFOSFOLIPIDOSJOSE LUIS ANACONAGEOVANNA ANDREA AYALA

MEDICINA Y CIRUGIAUNIVERSIDAD DEL VALLE

DEFINICIONHanly J. Antiphospholipid syndrome: an overview. CMAJ JUNE 24, 2003; 168 (13)

Giannakopoulos B. The Pathogenesis of the Anti phos pho lipid Syndrome. Nengl j med 368;11 march 14, 2013Trombofilia adquirida en la que los pacientes presentan trombosis y / o complicaciones vasculares en el embarazo atribuibles a insuficiencia placentaria, acompaados de pruebas de laboratorio que evidencian la presencia de anticuerpos antifosfolpidos en la sangre

The deep veins of the lower limbs and the cerebral arterial circulation are the most common sites of venous and arterial thrombosis, respectively.However, any tissue or organ vascular bed can be affected.

Antifosfolipidos : antiproteinas de union plasmatica.

Giannakopoulos B. The Pathogenesis of the Anti phos pho lipid Syndrome. Nengl j med 368;11 march 14, 20132

Ocurre en el 1 al 5% de la poblacin generalSu prevalencia se incrementa con la edad.Prevalencia del SAF secundario es del 30% (1 a 20 por cada 100,000 mujeres) Los aPL pueden estar presentes en el 50% de los pacientes afectados y en el 5% de la poblacin sana.> mujeres jvenes de edad frtil, solo el 12% de los pacientes superan los 50 aos

The syndrome occurs in isolation (primary antiphospholipid syndrome)or in association with connective tissue diseases (secondary antiphospholipid syndrome), particularly systemic lupus erythematosus.

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FISIOPATOLOGIA

Hanly J. Antiphospholipid syndrome: an overview. CMAJ JUNE 24, 2003; 168 (13)

Se desconoce el mecanismo que induce la produccion de anticuerpos, sin embargo se ha propuesto como evento desencadenant infeccion,

First, antiphospholipid antibodies may interfere with the function of the coagulation cascade leading to a procoagulant state.Examples include inhibition of the activated protein C and antithrombin III pathways, inhibition of fibrinolysis and upregulation of tissue factor activity. As previously mentioned, 2-glycoprotein I may function as an in-vivo anticoagulant and, thus, antibodies that targetthe molecule may interfere with this role. Other proteins that are important in regulating coagulation, such as prothrombin, proteins C and S, and annexin V, may also be targeted by antiphospholipid antibodies.Finally, there is evidence that the binding of annexin V to procoagulant surfaces may be inhibited by antiphospholipid antibodies.

Thus, antiphospholipid antibody binding tothe endothelial cell surface in a 2-glycoprotein-Idependent manner leads to endothelial cell activation, which is manifested by upregulation of cell surface adhesion molecules and increased secretion of interleukin-6 and prostaglandins.Activacion endothelial lleva a un estado procoagulante, sumado a un mecanismo de activacion plquetaria

el Segundo hit lleva a produccion de citoquimas y activacion endotelial4

CRITERIOS DIAGNOSTICOS

Hanly J. Antiphospholipid syndrome: an overview. CMAJ JUNE 24, 2003; 168 (13)

MANIFESTACIONES

Pueden ser una expresin directa o indirecta de la trombosis venosao arterial o morbilidad en el embarazo.Livedo reticulares : patrn moteado retculo vascular decoloracin purpura de la piel Probablemente es causada por la inflamacin de las vnulas, debido a la obstruccin de los capilares por trombos.6

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MANIFESTACIONES NEUROLOGICAS Manifestacin secundaria al proceso isqumico con compromiso del tejido cerebral o por accin directa de los anticuerpos anti fosfolpidos sobre el tejido neuronal.Yoav Arnson, MD. The Antiphospholipid Syndrome as a Neurological Disease. 2010 Elsevier Inc

Asociado a un amplio espectro de manifestaciones neurolgicas.Los sntomas neurolgicos pueden ser la caracterstica de presentacin de la enfermedad o pueden emerger durante el curso de la misma. El mecanismo por el cual hay compromiso del tejido nervioso es poco entendido.

It is well established that APS is associated with hypercoagulability . aPL antibodies can induce a prothrombotic state by activating platelets orendothelial cells by inhibiting protein C activation or by binding to phospholipids or2GPI on endothelial cells or platelet membranes resulting intheir activation . aPL also induce proinflammatory signals including nuclear factor-B and the p38 mitogen activation protein kinase . Neural injury maybe caused by thrombotic occlusion of brain vessels. 9

ECV -AITMigraaEpilepsiaDemenciaCorea y distoniaEncefalopata agudaDisfuncin cognitivaTrastornos psiquitricosEsclerosis mltipleMielitis transversaHipertensin intracraneal idioptica

MANIFIESTACIONES NEUROLOGICAS

ACV TIA

The Antiphospholipid Syndrome as a Neurological Disease

Son la tercera manifestacin mas comn despus de trombosis venosa y livedo reticularis. Y la principal manifestacin neurolgica.Sindrome de ac antifosfolipidos (APS) es una causa mayor y protencialmente prevenible de ACV.

Puede ocurrir como un episodio aislado o puede reaparecer.

The risk for recurrent stroke appears to be increased in active APS patients with multiple events, and particularly in a patient following the initial cerebral ischemic episode

La arteria cerebral media es la region mas afectada. Aunque los eventos isquemicos cerebrales pueden ocurrir encualquier region vascular.

La edad promedio de aparicion de los eventos isquemicos cerebrales asociados con aPL es aproximadamente una decada menor que la edad promedio de aparicion de isquemia para la poblacion general.

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Yoav Arnson, MD. The Antiphospholipid Syndrome as a Neurological Disease. 2010 Elsevier Inc

Existe una alta asociacin entre epilepsia y anticuerpos antifosfolipidos.Parcialmente se puede explicar por un insulto isqumico neuroparenquimatoso.La prevalencia de epilepsia posstroke en poblacin general es del 10 % mientras que en los pacientes consindrome de anticuerpos antfosfolipidos se estima de un 17% sugiriendo la interacion de otros factores como la interacion inmune directa con el cerebro.

aPL bind neurotransmitters such as ATP, thereby interfering with neuronal function . It has been suggested that aPL may have a direct effect on seizure genesis through inhibition of the gamma-aminobutyric acid receptor-ion channel complex, which in turn may increase neuronal excitability.12

Yoav Arnson, MD. The Antiphospholipid Syndrome as a Neurological Disease. 2010 Elsevier Inc