Pediatric Rheumatology Online Journal → July 2003 → Anti-Phospholipid Antibody Syndrome→ Abstract #114
PRIMARY AND SECONDARY ANTIPHOSPHOLIPID SYNDROME (APS) IN CHILDREN AND ADOLESCENTS
S. Garay,1 F. Perez,1 D. Giacomone,1 R. J. Cuttica,1 A. Pringe,1 M. I. Brusco.1
Antiphospholipid syndrome has been clearly defined; the aim of this study was to analyze prognosis, clinical and serologic features of 17 pediatric patients with primary and secondary APS. This is an observational retrospective study that included 6 patients with primary and 11 secondary APS. Sapporo1998 criteria were used and for the diagnosis of disease associated, usual criteria for each of them. Anticardiolipin antibodies were determined by ELISA and Lupus Anticoagulant according to the Thrombosis and Hemostasia Society .Six patients had Primary APS, 3 were girls, mean age 1.9 years (Mn5 months-Mx 52 months); 5/6 had ischemic stroke and 1 deep venous thrombosis in upper and lower limbs, this patient died because of pulmonary thromboembolic event; patients with stroke had favorable outcome. All of them had positive aCL, LAC was positive in 1/3 and B2GP1 in 2/3. Secondary APS was seen in 11 patients (5 SLE, 2 SLE like, 2 MCTD and 1 varicella and 1 HIV infections) 8 were girls and 3 boys. Mean age was 12.2 years (Mn1.6 y-Mx 22 y). Four out of 11 patients developed the syndrome at the same time of associated disease onset, the others after a mean time of 3.9 years. Eight out of 11 patients had other clinical findings associated to APS such as autoimmune hemolytic anemia 3, Evans syndrome 1, livedo reticularis 5 and digital necrosis or skin ulcers 2 patients. Three patients died, 2 because of pulmonary thromboembolism and multiorgan failure. Ten patients had positive aCL (3 SLE and 2 infections); B2GP1 was positive in 4/4 and LAC was positive in 5/7.Conclusion: 1-Primary APS were predominantly ischemic stroke (83%, p=0.04) without associated clinical findings and onset at early age. 2- Secondary APS had deep venous thrombosis, arterial thrombosis, pulmonary thromboembolism and stroke and was associated to hemolytic anemia, thrombocitopenia, transverse myelitis, livedo reticularis, digital necrosis and skin ulcers; syndrome onset was in elder patients.