Malattia di Behçet: una sfida per il pediatra

Abstract

Behçet’s disease (BD) is a rare chronic inflammatory condition associated with variable-vessel vasculitis, systemic involvement and relapsing course. It is characterized by a wide clinical heterogeneity, presenting with mucocutaneous, ocular, articular, gastrointestinal, neurological and vascular manifestations in the long term, with a high risk of delay in diagnosis: therefore, its morbidity and mortality are significant. The definition of BD probably encompasses a range of disorders with unclear pathogenesis, overlapping autoinflammatory and autoimmune pathological mechanisms. Diagnosis is clinical and multiple classification criteria have been developed. The progressive knowledge about its molecular basis, especially in the so-called monogenic mimics of BD, resulted in an improvement in the disease therapy, with an optimization of the use of conventional immunosuppressive agents and the introduction of new biologic drugs. BD occurs in childhood in up to 20% of cases: paediatric BD differs from adult BD in frequency and severity of clinical findings, and, above all, for a frequent incomplete clinical picture, making the recognition and management of the disease a challenge. The new paediatric classification criteria are aimed to allow an early diagnosis and a proper therapy.