Article information
Abstract
The classical clinical picture of the antiphospholipid syndrome (APS) is characterized by venous or arterial thromboses, fetal losses and thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant, anticardiolipin antibodies or antibodies directed to various proteins, mainly β2 glycoprotein I, or all three. Apart from being “primary” (without any discernable underlying systemic autoimmune disease), or associated to another disease (usually to systemic lupus erythematosus), it may also occur rapidly over days or weeks when it has been termed “catastrophic” APS. Therapy should not primarily be directed at effectively reducing the aPL levels and the use of immunotherapy (including high dose steroid administration, immunosuppression or plasma exchange) is generally not indicated, unless in the catastrophic APS. Treatment of APS patients should be based on the use of antiaggregant and anticoagulant therapy.
Keywords:
Antiphospholipid syndrome
Antiphospholipid antibodies
Anticardiolipin antibodies
Lupus anticoagulant
Resumen
El síndrome antifosfolipídico (SAF) se caracteriza por la asociación de los anticuerpos antifosfolipídicos (AAF) con trombosis de repetición, abortos o pérdidas fetales recurrentes y trombocitopenia. Los AAF más estudiados son los anticuerpos anticardiolipina, el anticoagulante lúpico y los anticuerpos anti-β2-glucoproteína I. El SAF puede presentarse de forma aislada, denominándose SAF primario, o bien asociado a otras enfermedades autoinmunes sistémicas, fundamentalmente a lupus eritematoso sistémico. Más recientemente, se ha descrito un subgrupo de SAF en el que los pacientes desarrollan múltiples trombosis durante un corto espacio de tiempo, que se ha denominado SAF catastrófico. Aunque parece clara la asociación entre la presencia de AAF y trombosis, la actitud terapéutica no debe ir dirigida primariamente a la eliminación o a la reducción de los niveles de estos anticuerpos mediante recambios plasmáticos, gammaglobulinas intravenosas o inmunodepresores (excepto en el SAF catastrófico), y a que no existe una clara correlación entre los niveles de los AAF y los episodios trombóticos. El tratamiento de estos pacientes debe basarse en el uso de antiagregantes plaquetarios o anticoagulantes.
Palabras clave:
Síndrome antifosfolipídico
Anticuerpos antifosfolipídicos
Anticuerpos anticardiolipina
Anticoagulante lúpico
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