Behçet’s Disease In Children And Adults Of Sub-Saharan Ancestry: A Systematic Review And Meta-Analysis

TL;DR

Behçet’s disease in people of Sub-Saharan ancestry is more severe and has unique features, especially involving the central nervous system.

Contribution

This study provides the first systematic review and meta-analysis of Behçet’s disease in individuals of Sub-Saharan ancestry.

Findings

Behçet’s disease in sub-Saharan ancestry individuals is predominantly HLA B51/B5-negative.

Central nervous system involvement is common and severe, with high rates of cerebrovascular disease and parenchymal lesions.

Most patients achieve remission, but many suffer severe long-term effects, particularly in the eyes and nervous system.

Abstract

The characteristics of Behçet’s disease (BD) in individuals of Sub-Saharan ancestry (sub-SA) are poorly understood.

Herein, we conducted a PRISMA-compliant systematic review using the PubMed/Medline, Scopus, and Web of Science databases. Articles published up to September 1, 2023 were searched with the following keywords: "Behçet's disease" OR "Behcet's syndrome" AND "sub-Saharan African" OR "Black" OR “African”. Data on the year, type and country of study, sample size, region of origin, nationality, age, sex, time to diagnosis, death, HLAB51 status, mucosal, and organ involvement were collected. Involvement of the central nervous system was retained on the basis of objective criteria, and dichotomized into parenchymal or non-parenchymal/vascular lesions. The pooled frequency of patients’ main characteristics was calculated using a DerSimonian-Laird random-effects meta-analysis.

This study included 42 full-text reports, with study periods ranging from 1970 to 2023. Overall, 230 adult patients (69% of males) were included, of whom 195 (85%) were from sub-Saharan African countries, 22 (10%) patients were from the Caribbean, and 13 (5%) patients were from the Americas, including 12 African Americans, and 1 African Brazilian. Oral and genital ulcers were reported in 98% [95% CI 91 to 100%] and 85% [72 to 92%], respectively. Ocular involvement occurred in 43% [31 to 56%] of patients. Central nervous system (CNS) involvement affected 39% [25 to 54] of the patients. Among them, 30% of patients had a cerebro-vascular disease, and 72% had a parenchymal involvement. The patients were mostly treated with oral steroids and colchicine, and remission was achieved in 35/54 (65%) patients, but 15 (69%) of them suffered severe sequelae, particularly ophthalmological and neurological.

Behçet’s disease in patients of sub-Saharan ancestry appears to be predominantly HLA B51/B5-negative, and more severe than in other ethnicities, owing to a high prevalence of CNS involvement.

The online version contains supplementary material available at 10.1007/s12016-025-09085-8.

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