Behçet's disease in Portugal

Area of Science:

• Rheumatology
• Immunology
• Ophthalmology

Background:

• Behçet's disease is a rare multisystemic inflammatory disorder.
• Historical data on Behçet's disease in Portugal is limited.
• Previous reports indicated a low prevalence until the late 20th century.

Purpose of the Study:

• To analyze the epidemiological and clinical characteristics of Behçet's disease in Portugal.
• To report the incidence and prevalence of the disease across different regions of Portugal.
• To document treatment modalities and outcomes in Portuguese patients.

Main Methods:

• Retrospective analysis of 156 diagnosed patients with Behçet's disease in Portugal.
• Review of patient records from various regions including Lisbon, Oporto, and Coimbra.
• Inclusion of data on clinical manifestations, HLA-B5 association, and treatment regimens.

Main Results:

• 156 patients diagnosed over several decades, with a significant increase in diagnoses in recent years.
• High incidence of oral ulcerations (100%) and ocular involvement (87%) observed.
• HLA-B5 association found in 50% of patients; mortality rate of at least 4 cases, with 3 due to neuro-Behçet.

Conclusions:

• Behçet's disease is present in Portugal with characteristic multisystemic involvement.
• Ocular and oral manifestations are highly prevalent.
• Standard and advanced therapies were employed, with notable cases of neuro-Behçet and fatalities.