b2GP-I in the anti phospholipid (Hughes') syndrome—from a cofactor to an autoantigen—from induction to prevention of antiphospholipid syndrome

Y Shoenfeld, A Gharavi, T Koike - Lupus, 1998 - journals.sagepub.com
Y Shoenfeld, A Gharavi, T Koike
Lupus, 1998journals.sagepub.com
The Hughes' syndrome (anti-phospholipid) consists of the triad of recurrent venous and
arterial thrombosis, fetal loss and thrombocytopenia. 1±4 Since it was first reported in 1983,
1, 3 its clinical presentation has expanded to include heart involvement (Libman-Sacks'
endocarditis) 4, 5 central nervous system expression4, 6, 7 and other skin and blood
findings. 4, 8 The close association of the clinical presentation with high titers of IgG anti-
cardiolipin antibodies and the presence of lupus anticoagulant established its name as the …
The Hughes' syndrome (anti-phospholipid) consists of the triad of recurrent venous and arterial thrombosis, fetal loss and thrombocytopenia. 1±4 Since it was first reported in 1983, 1, 3 its clinical presentation has expanded to include heart involvement (Libman-Sacks' endocarditis) 4, 5 central nervous system expression4, 6, 7 and other skin and blood findings. 4, 8 The close association of the clinical presentation with high titers of IgG anti-cardiolipin antibodies and the presence of lupus anticoagulant established its name as the anti-phospholipid syndrome (for example, anti-phosphatidyl serine,(anti-PS) anti-prothrombin). Later on, other anti-phospholipid autoantibodies were found to be related to its expression. 9 The direct pathogenic role of the anti-phospholipid autoantibodies (for example, anti-cardiolipin and anti-PS) was established in 1991 when, following passive infusion of monoclonal and polyclonal mouse and human aCL10, 11 and aPS, 12 to naive BALB/c mice, the fully expressed syndrome was induced. 13±15 These results were further supported by induction of experimental APS in mice following active immunization with the respective autoantibodies. 16, 17 A summary of the importance of idiotypes in APS was published. 17
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