Prognosis in Behçet’s disease: what to expect?

Abstract

The prognosis of Behçet’s disease is intricately shaped by various factors. Male gender and younger age (20–40 years) are unequivocally linked to ocular manifestations, large vessel involvement, and increased mortality. Worse outcomes are associated with major organ involvement, such as vascular disease, blindness-inducing eye involvement, and neurological complications, contributing to elevated morbidities. Pulmonary artery aneurysms, identified as a primary cause of mortality, are specifically associated with a significant 50% mortality rate within 1 year. Early treatment initiation is underscored for more favorable outcomes. Alongside pulmonary artery aneurysms, leading causes of mortality encompass central nervous system (CNS) involvement, gastrointestinal involvement, Budd–Chiari syndrome, heart issues, renal complications and cancer. Studies among pediatric disease align with adult studies emphasizing the crucial roles of ocular and neurological involvements in determining outcomes. Advanced age correlates with a decrease in disease activity, and patients presenting with mucocutaneous manifestations often experience a diminishing disease activity over time.