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

Abstract

Introduction: Antiphospholipid syndrome (APS) is an autoimmune, multisystemic disease characterized by the presence of thrombotic events, gestational morbidity, as well as hematological, dermatological and neurological manifestations, in the presence of high titers of antiphospholipid antibodies. It can present as primary, an isolated clinical entity, or secondary, as a manifestation of a rheumatologic disease, primarily systemic lupus erythematosus. 35 years after its first description, the understanding of this pathology is still evolving and even more so in the presentation in children. Objective: To do a demographic description of pediatric population with APS in our hospital, allowing us to establish diagnostic criteria and targeted therapeutic management. It will also allow a prompt diagnosis, avoiding the morbidity and mortality associated with this pathology. Materials and Methods: We performed a descriptive cross-sectional study. We searched in our archives for patients under 16 years of age, evaluated at the Hospital Infantil de Mexico Federico Gomez from March 2000 to March 2018 that met Sapporo criteria for classification of antiphospholipid syndrome. Results: By reviewing the archives, we documented 29 patients were met Sapporo criteria for classification of APS from March 2000 to March 2018 at Hospital Infantil de Mexico Federico Gomez. 52% of the patients were female, while 48% male. The average age of patients was 9.8 years, with a minimum of 2.2 and a maximum of 16 years. The mean age at diagnosis was 14.8 years, with a standard deviation of 4.56 (range from 6.2 to 14.2 years). Of the 29 patients, 48% were diagnosed with primary APS, while 52% with secondary. Systemic Lupus Erythematosus was the primary rheumatologic disease in all the cases of secondary APS. Conclusion: Antiphospholipid syndrome is a complex and not well described pathology in pediatric population. Its variable presentation and unpredictable nature implies a diagnostic and treatment challenge for pediatricians. Epidemiological description of the pediatric population with APS at our hospital is very valuable for the development of diagnostic and therapeutic guidelines. Future reviews, in correlation with reviews from tertiary international rheumatologic centers are needed.