[Clinico-immunological aspects of antiphospholipid syndrome]

The article contains a description of the clinical-and-immunological specific features of the secondary (concomitant with systemic lupus erythematosus-SLE) and primary anti-phospholipid syndrome (APS). We followed up a total of 280 patients with SLE and 74 patients with primary APS; 96 of 280 SLE patients were males and 184 of them were females, mean age--31.2 +/- 11.1, mean disease duration--8.6 +/- 7.2 years. The APS group consisted of 15 males and 59 females, mean age--35.6 +/- 7.2, mean disease duration--11.9 +/- 8.5 years. Antibodies to cardiolipin (aCL) and lupous anticoagulant (LA) were determined for all patients. Antibodies to IgM beta 2-GP I (IgM anti-beta-GP I) were determined for 102 patients and antibodies to IgG anti beta 2-GP I were determined for 153 patients. The level of antibodies to beta 2 GP I in blood serum was found by immune-enzyme assay (QUNTA Life). The secondary APS was diagnosed in 115 (41%) patients from among 280 SLE patients. The group of patients with SLE and without APS and the group with primary APS were age-matched, however, patients with APS were older and had a longer disease duration. The diagnosis was made in one half of patients with primary APS in 6 and more years after the disease onset. Thrombotic complications were found in 117 (42%) from among 280 SLE patients. They were encountered reliably more often in APS versus SLE (chi 2 = 131, p < 0.0001). Venous thrombosis in veins of lower extremities prevailed in APS, while thrombophlebitis of the surface shin veins was predominant in patients without APS. An important features of APS is occlusion of veins located in the liver with a subsequent development of Budd-Chiari syndrome (9 patients); in retinal veins (13 patients); thrombophlebitis in axillary veins (7 patients) and in abdominal-cavity veins (2 patients). Loss of pregnancy at various gestational stages was registered in medical histories of 83 (76%) from among 109 pregnant women. The frequency rate of fetus loss was higher in primary APS (32 of 40 cases) versus its secondary variation (49 of 75 cases) with chi 2 = 8.3, p = 0.004. Thrombocytopenia was detected reliably more often in patients with secondary APS (chi 2 = 36, p < 0.0001). The clinical signs of both primary and secondary APS were found to be similar. However, arterial and venous thromboses combined with the relapsing syndrome of fetus loss were more often registered in primary APS. The detection of highly positive aCL titers is of a significant diagnostic value versus the detection of its low- and mean-positive values. The study of anti-beta 2-GP I is also of certain importance for a more reliable diagnosis of APS.