Enfermedad de Behçet en pediatría, un reto diagnóstico: revisión sistemática cualitativa de la literatura

Abstract

Behçet's disease is a systemic inflammatory disease that mainly affects the oral and genital mucosa, skin and eyes. A qualitative systematic review of the literature is carried out in search of finding the epidemiology, etiopathogenesis, spectrum of clinical manifestations, diagnosis, treatment approach and current Clinical Trials of Behçet's disease in the pediatric population. A bibliographic search was performed in PubMed without language or publication date restrictions. References of included articles were examined for additional relevant literature. The initial search yielded a total of 570 studies from PubMed, one from a website, 4 from the Colombian Ministry of Health and 4 from a review of bibliographic citations, of which 32 articles were included for the present review, finding that the prevalence at a global level it is estimated around 10.3 per 100,000 inhabitants. Behçet's disease is a vasculitis that affects vessels of all sizes including veins. It was recently reclassified as variable type vasculitis. Other frequent clinical manifestations are joint, skin and digestive manifestations. Although its etiopathogenesis is not clear, in recent years it has been considered a multicausal autoinflammatory entity. Its diagnosis is mainly clinical. Management should be individualized based on the manifestations of the disease given the clinical variability. Multicenter, placebo-controlled, standardized studies that involve large series of patients, use clinical scores, and have long-term follow-up are needed to better understand the nature of this disease.