The
antiphospholipid syndrome (APS) is defined by the occurrence of venous and arterial
thromboses and recurrent fetal losses, frequently accompanied by a moderate
thrombocytopenia, in the presence of
antiphospholipid antibodies (aPL), namely
lupus anticoagulant (LA),
anticardiolipin antibodies (aCL), or anti-β2
glycoprotein-I (β2GPI)
antibodies. The current mainstay of treatment for thrombotic APS is
heparin followed by long-term anticoagulation, while in obstetric APS, the accepted first-line treatment consists in low-dose
aspirin (LDA) plus prophylactic unfractionated or
low-molecular-weight heparin (
LMWH). Recently, new emerging treatment modalities, including
intravenous immunoglobulins (
IVIG), have been implemented to manage APS refractory to conventional
therapy. The objective of this review is to summarize the currently available information on the
IVIG therapy in APS, focusing on the use of
IVIG in the obstetric form, CAPS and on primary or secondary thromboprophylaxis. We analyzed 35 studies, reporting the effects of
IVIG in APS patients, and we discussed their results.
IVIG in obstetric APS seem to be very useful in selected situations (patients not responsive to the conventional treatment, concomitant autoimmune manifestations or
infections or patients in whom anticoagulation is contraindicated).
IVIG treatment represents an important component of the combination
therapy of CAPS and they could be useful, in addition to the standard
therapy, to prevent recurrent
thrombosis in APS patients refractory to conventional
anticoagulant treatment. Anyway, in some cases we also found controversial results that claim the need of further well-designed studies to definitely state the efficacy and tolerability of
IVIG in CAPS, obstetric and non-APS.