The study of antiphospholipid antibodies (aPL), not included in the Sydney diagnostic criteria, in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) is poorly understood.The aim of this study – to determine the clinical significance of IgG antibody testing for domain I β2 -glycoprotein 1 (β2 -GP1DI) – IgG anti-β2 -GP1DI in patients with APS with and without SLE.Materials and methods. The study included 187 patients with APS with or without SLE, 49 patients formed a comparison group, and 100 relatively healthy individuals formed a control group. IgG/IgM antibodies to cardiolipin and IgG/ IgM anti-β2 -GP1 were determined by enzyme immunoassay (ELISA) in patients with or without APS, and IgG antiβ2 -GP1DI was determined by chemiluminescence assay in all patients and controls.Results. IgG anti-β2 -GP1DI was detected in 37 (71%) of 52 patients with primary APS (PAPS), in 6 (50%) of 12 patients with probable APS, in 42 (71%) of 59 patients with SLE+APS, in 17 (26%) of 64 patients with SLE, in 1 (2%) of comparison group and in none of control group. IgG anti-β2 -GP1DI was significantly associated with PAPS and SLE+APS compared with patients with SLE (p=0.0002 and p=0.0001, respectively). The association of IgG anti-β2 -GP1DI with clinical manifestations of APS (thrombosis (χ2 =9.69; p=0.001) and obstetric pathology (χ2 =4.19; p=0.04)) was detected. There was a significant association of IgG anti-β2 -GP1DI with arterial thrombosis (χ2 =8.84; p=0.002) and with late gestational obstetric pathology (χ2 =6.35; p=0.01). High specificity of IgG anti-β2 - GP1DI depending on the diagnosis and clinical manifestations of APS was noted despite low sensitivity: specificity for thrombosis was 84%, for obstetric pathology – 94%, for APS – 89%. Isolated IgG anti-β2 -GP1DI positivity was reported in 2% of 50 aPL negative patients and was not associated with APS manifestations.Conclusion. The frequency of IgG anti-β2 -GP1DI detection was higher in patients with APS compared to patients with SLE, comparison group and control (p<0.05). Positive IgG anti-β2 -GP1DI values were significantly associated with thrombotic complications and with obstetric pathology (χ2 =8.84; p=0.002 and χ2 =6.35; p=0.01). Specificity>˂ 0.05). Positive IgG anti-β2 -GP1DI values were significantly associated with thrombotic complications and with obstetric pathology (χ2 =8.84; p=0.002 and χ2 =6.35; p=0.01). Specificity of IgG anti-β2 -GP1DI for APS and its clinical manifestations (thrombosis and obstetric pathology) was higher than sensitivity: 89%, 94%, and 84%, respectively.