Background A clinical polymorphism of systemic lupus erythematosus (SLE) encourages the search for early predictors of its flow. Detection of SLE with antiphospholipid syndrome (APS) in children has an important prognostic value, as it implies a high risk of thrombosis, and also determines the course and outcome of the underlying disease.
Objectives Evaluate the clinical features of systemic lupus erythematosus with APS in children.
Methods The basis of this work was aimed to survey results and data archival materials of 153 SLE patients observed in the clinic of the Scientific Center of Pediatrics and Pediatric Surgery the Republic of Kazakhstan. Determination of antibodies to cardiolipin (aCL) was carried out by ELISA. Detection of lupus anticoagulant (LA) was held in phospholipids-dependent tests of blood clotting in plasma that had shortage in platelets. All samples were tested in duplicate.
Results A study group consisted of 40 (26.1%) SLE patients with different clinical manifestations of APS. The first manifestations of APS in most patients arise during relapses (64.7%). In 17.6% of patients symptoms of APS were observed during the first 2 - 4 weeks from the beginning of SLE. In 11.8% of cases, clinical manifestations of SLE were preceded by APS. Less commonly APS developed during remission (5.9%).
Thrombosis in children with SLE were mainly venous thrombosis: small vessels of the skin (27.3%), presented by palm and plantar erythema, purpura, skin surface necrosis, deep vein thrombosis of shins, that 3 children had. In two (9.1%) of 22 patients there was thrombosis of the central vein of the retina. One girl with arterial thrombosis CNS (13 years old, the duration of the disease: 6 years), was diagnosed with transient ischemic attacks in the form of convulsions, memory impairment. Cytopenia determined in a half (59.0%), as a mild thrombocytopenia (34.4%). The combination of hemolytic anemia and thrombocytopenia was observed in one case (4.5%). Valvular heart disease (hardening of the valves, vegetation, mitral insufficiency and tricuspid valves II degree) were detected equally often - in 36.4% of cases. Neurological symptoms was determined in 4 (18.2%) children. The main symptoms were headache by type of migraine (13.6%) and in one case - episyndrome (4.5%). Aseptic necrosis of bone were found in 2 (9.1%) patients. One was recorded with necrosis of the femoral head, other with bone necrosis of the nasal septum. Among other manifestations of APS arterial hypertension (AH) had an essential part, which was observed in 32.5% of patients. A frequent combination (84.6%) of hypertension with signs of kidney damage should also be noted.
In patients with secondary APS degrees of the main factors of hemostasis were significantly higher than in patients without APS, which explains the severity of the conditions and vascular disorders that were detected in the studied children.
Conclusions Early diagnosis and aggressive treatment is pathogenetically substantiated SLE patients with APS will help achieve positive outcomes.
Disclosure of Interest None declared