Abstract
ObjectiveDescribe the frequency of thrombotic and non-thrombotic clinical manifestations, laboratory, treatment and prognosis in patients with pediatric primary antiphospholipid syndrome.Material and methodsA retrospective study was carried out in patients with a diagnosis of primary antiphospholipid antibody syndrome, under 16 years of age, under follow-up by the pediatric rheumatology service of the General Hospital, National Medical Center, La Raza, from January 2013 to December 2020. The antiphospholipid syndrome was defined when it met the laboratory criteria of the Sidney criteria and the presence of thrombosis or non-criteria manifestations of the disease (hematological, neurological, cutaneous, renal, cardiac or pulmonary). Demographic, clinical, laboratory, treatment, and prognosis data were collected.ResultsWe report 32 patients, 21 female (65%) and 11 male (35%), mean age 11.75 years, evolution time 16 weeks. Thrombosis 9 patients (28%), 1 arterial and 8 venous. Non-thrombotic manifestations; Hematologic: thrombocytopenia 22 patients (69%), autoimmune hemolytic anemia 13 (40%), Fisher-Evans syndrome 6 (19%), lupus anticoagulant with hypoprothrombinemia syndrome 2 (6%). Dermatological: livedo reticularis 20 (62%), skin ulcers 2 (6%), Raynaud's phenomenon 8 (25%). Neurological: epilepsy 1 (3%), migraine 3 (9%), chorea 1 (3%) and cognitive impairment 3 (9%). Renal in 4 (13%). Laboratory: prolonged aPTT 30 (93%), lupus anticoagulant 32 (100%), positive IgG anticardiolipin 20 (62%), positive IgM anticardiolipin 19 (60%). AntiB2GPI was performed in only 3 patients, being positive in all. Treatment: anticoagulation in patients with thrombosis, antiplatelet in 23 (72%), steroid 30 (94%), immunosuppressant 30 (94%) and rituximab 4 (12.5%). No deaths were reported.ConclusionsThe clinical characteristics of patients with pediatric primary antiphospholipid syndrome differ from those presented in adults, since non-thrombotic manifestations are more frequent in children, for which classification criteria that include these manifestations are necessary for a better characterization of the disease in pediatric population.
Highlights
Antiphospholipid syndrome is an acquired immune mediated prothrombotic state
The clinical characteristics of patients with pediatric primary antiphospholipid syndrome differ from those presented in adults, since non-thrombotic manifestations are more frequent in children, for which classifica‐ tion criteria that include these manifestations are necessary for a better characterization of the disease in pediatric population
In 1952 researchers from Johns Hopkins University reported an approximate incidence of false positive Venereal Disease Research Laboratory (VDRL) in 20% of patients with lupus, which was later associated with the presence of lupus anticoagulant
Summary
Antiphospholipid syndrome is an acquired immune mediated prothrombotic state. It is an important cause of thrombosis and obstetric loss. The history of this condition can be traced back to observations made during syphilis detection programs carried out in the first half. In 1952 researchers from Johns Hopkins University reported an approximate incidence of false positive VDRL in 20% of patients with lupus, which was later associated with the presence of lupus anticoagulant. Later in 1983, Harris used radioimmunoassay to show that at least two-thirds of serum samples from a group of 65 lupus patients had elevated levels of anticardiolipin antibodies. 90% of these patients who had lupus anticoagulant positivity had elevated levels of anticardiolipin antibodies [1, 2]
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