Antiphospholipid antibodies and the antiphospholipid syndrome: an update on treatment and pathogenic mechanisms.
OBJECTIVE
The antiphospholipid syndrome is a disorder of recurrent thrombosis, pregnancy loss and thrombocytopenia associated with the presence of antiphospholipid antibodies and persistently positive anticardiolipin or lupus anticoagulant positive tests. Since its recognition in the 1980s, growing interest in the field, not only with respect to diagnosis and treatment, but also regarding the pathogenesis of antiphospholipid antibodies, has emerged.


RESULTS
First, this review addresses the recently updated classification criteria for diagnosis and treatment of the antiphospholipid syndrome. A discussion on the newly described potential beneficial roles of hydroxychloroquine and the statins for the treatment of antiphospholipid syndrome-associated clinical manifestations is included. Importantly, this article analyzes recent data that examine the molecular and intracellular events that antiphospholipid antibodies trigger in target cells, as well as new findings in the identification of the receptors for these antibodies on the membrane of those cells. A separate section discusses novel pathogenic mechanisms of antiphospholipid antibodies, including the activation of complement and their interaction with homologous catalytic domains of several serine proteases of the coagulation system.


CONCLUSIONS
Understanding the molecular interactions and the intracellular signaling that antiphospholipid antibodies trigger, new therapeutic and targeted strategies to ameliorate clinical manifestations in patients with antiphospholipid syndrome may be established.
Pierangeli
Silvia S
SS
Department of Microbiology, Biochemistry and Immunology. Morehouse School of Medicine, Atlanta, GA 30310 1459, USA. spierangeli@msm.edu
Chen
Pojen P
PP
González
Emilio B
EB
D017152:Antibodies, Antiphospholipid / Q000276:immunology*; D000925:Anticoagulants / Q000008:administration & dosage; D016736:Antiphospholipid Syndrome / Q000175:diagnosis / Q000188:drug therapy; D001792:Blood Platelets / Q000276:immunology; D003167:Complement Activation / Q000276:immunology; D042783:Endothelial Cells / Q000276:immunology; D006801:Humans; D013927:Thrombosis / Q000188:drug therapy / Q000209:etiology
D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants