Clinical Features, Diagnosis and Treatment of Pediatric Behcet and Late-Onset Behcet Disease

Behçet disease is a multisystemic inflammatory vasculitis that is frequently observed in young males. Its clinical findings and also presenting age are quite heterogenous. Patients under the age of 16 years classified as pediatric Behçet s disease and those over the age of 50 classified as late-onset Behçet s disease demonstrate the most discrepancy regarding clinical presentation and prognosis and thus compose the patient group with delayed diagnosis and improper treatment. Pediatric Behçet disease is usually seen between the age of 7-13 and reported to be 7-26% of Behçet s cases. More frequent family history, female predominance in some regions, low ocular involvement are among the distinctive features. Regarding this fact, an international consensus group developed new diagnosis criteria. Ocular involvement is bilateral and in the form of panuveitis as in classical form. Cataract, maculopathy, and optic atrophy are among the most frequent complications. Late-onset Behçet disease presents usually between the ages of 47-54 and in males. Diagnosis criteria are the same as the young adult group however ocular involvement is lower and frequently in the form of anterior and panuveitis. Cataract and optic atrophy are among the most frequent complications leading to low vision. In this review, similar and distinctive features of the classical form observed in young adults and pediatric or late-onset Behçet s cases are scrutinized in the context of existing literature aiming to decrease the defects in diagnosis and treatment.