The varicella-autoantibody syndrome

This cross-sectional study was conducted to determine the incidence of auto antibodies to phospholipids and coagulation proteins in children with acute varicella zoster virus (VZV) infection. Study groups included children wit h VZV alone or complicated by purpura fulminans and/or thromboembolism. VZV naive children and children who had VZV > 1 y before sample collection for med a control group. Blood was assayed for the following: free protein S (P S), protein C, antithrombin, and prothrombin; antibody binding to these pro teins; lupus anticoagulant; anticardiolipin antibody; antiphospholipid anti bodies; and prothrombin fragment 1+2. Data regarding coinfections was colle cted. Forty-three VZV-infected children showed an increased frequency of lu pus anticoagulant, anticardiolipin antibody. antiphospholipid antibodies, a nd autoantibodies to PS, protein C, prothrombin, and antithrombin in compar ison to 52 children without acute VZV (p < 0.0001). Seventeen children with VZV and purpura fulminans and/or thromboembolism showed a statistically si gnificant decrease in free PS, significantly increased PS IgG antibody, and significantly increased prothrombin fragment 1+2 (p < 0.0001) compared wit h the group without acute VZV and the group with uncomplicated VZV. Twenty- six children with uncomplicated VZV showed increased PS IgG antibody (p < 0 .001) compared with the children without acute VZV. For all groups combined , elevated PS IgG antibody showed negative correlation with free PS (p < 0. 0001) and positive correlation with prothrombin fragment 1+2 (p = 0.0002). Autoantibodies were transient. Transient antiphospholipid and coagulation p rotein autoantibodies were common with VZV infection, but were not predicti ve of thrombotic complications.