Antiphospholipid syndrome in Mexican children: evolution, laboratory and clinical characteristics: a 10-year experience

Abstract

Introduction: The antiphospholipid syndrome (APS) is a multisystem and autoimmune disease, which is mainly characterized by the presence of thrombotic events, gestational morbidity, in the presence of high titers of antiphospholipid antibodies. It can present as a primary entity, or secondary to another autoimmune disease. The understanding of this pathology is still evolving and even more in its presentation in the pediatric patients. The presence of antiphospholipid antibodies has been widely reported in pediatric patients with thrombosis. APS is considered the most common acquired cause of a prothrombotic state. At the moment, there are no reliable data of its presentarion in pediatrics. The long-term morbidity and mortality associated with thrombosis events in pediatric population may be minored by determining prognosis and select patients for prophylactic treatment and more rigorous follow-up. Objective(s): To describe the clinical presentation and evolution, in addition to laboratory findings, in Mexican pediatric population with diagnosis of APS. Identified patients with arterial or venous thrombosis and its relation with laboratory findings. Describe epidemiology of Mexican pediatric population with APS. Method(s): Retrospective cohort study of the Children's Hospital of Mexico Federico Gomez, last 10 years. We reviewed the data form the clinical archives of the patients with diagnosis of APS according to the Miyakis criteria, from 2007 to 2017. The variables analyzed include age at diagnosis, sex, subtype of APS, clinical finding of thrombosis, laboratory finding of antiphospholipid antibodies and outcome. Result(s): A total of 29 patients fulfil the diagnosis criteria of APS. The mean age of patients at diagnostic was 9.8 years, with a minor age at diagnosis of 2.2 years and a maximum of 16.4 years of age. Of the total population 52% were females and 48% males. Primary APS was diagnosed in 48%, of which 71% were males. Secondary APS was present in 52% of the population, all diagnosed with Systemic Lupus Erythematosus. Of the patients with secondary APS, 85% were female. Arterial thrombosis was present in 48% of the cases, primarily in the group of secondary APS. Of the total cases 20% presents with a second thrombosis event despite anticoagulant therapy, of this 66% were diagnosed with secondary APS. Lupus anticoagulant (LA) was present in high titers in 93% of the patients, while anti-B2 glycoprotein-1 antibody IgG was present in 27% and IgM in 31%. Anticardiolipin antibody IgG was positive in 17% of the population, while isotype IgM in 24%. All patients receive long term anticoagulant therapy. One male patients, with secondary APS developed catastrophic APS. One patient required of amputation for deep venous thrombosis, and another patient died as a complication of gastrointestinal thrombosis, both males with primary APS. Conclusion(s): During a 10 year follow up, we diagnosed 29 patients with APS. As reported in literature, a greater percentage of patients are female and present with a secondary APS. Most patients with primary APS are males. In our population, in contrast with what literature report, secondary APS presents with a greater percentage of arterial thrombosis. The mayor site of venous thrombosis was low extremities and of arterial thrombosis was cerebrovascular events. The presence of positive LA implicates a higher risk of thrombosis. Our pediatric population diagnosed with APS requires of a close follow up in order to monitor anticoagulant therapy and to prevent the patients from developing a second thrombotic event that may lead to death.