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

Abstract

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

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: Behçet’s disease; Black; Ethnicity; Meta-analysis; Sub-Saharan ancestry; Systematic review.

Fig. 1

Study flow diagram according to PRISMA guidelines of the selection and inclusion of articles in the systematic literature review on Behcet’s disease in children and adults of sub-Saharan ancestry. * Literature analysis was performed again on the 29.^th November 2024, but identified no further relevant articles (n = 6 new articles found)

Fig. 2

Summary of the distribution of Behcet’s disease manifestations in adult individuals of sub-Saharan ancestry. NB: Detailed percentages of neurological and ocular damage are given for patients affected with these condition. The illustration has been created with Biorender.com®

Fig. 3

Overview of the geographic distribution of Behçet’s disease (BD) in individuals of sub-Saharan ancestry (sub-SA), showing the number of patients per country and summary of characteristics of patients of sub-SA with BD in the Americas, Caribbean, and sub-Saharan Africa regions. The Americas comprised patients from USA and Brazil. The Caribbean comprised patients from Guadeloupe, Jamaica, and Haiti. The sub-Saharan Africa comprised the other countries. Data of patients from Spain [72] Egypt [73] Turkey [74] and Japan [75] are given for comparative purpose. CNS: central nervous system involvement; GU: genital ulcers; OU: oral ulcers. *The country was unknown for 4 African Caribbean patients and for 52 sub-Saharan African patients. ** data available for 9 African Caribbean patients and for 177 sub-Saharan African patients

Fig. 4

Pooled frequencies of adult individuals of sub-Saharan ancestry with Behçet’s disease meeting ISG (a) and ICBD4 criteria (b)

Fig. 5

Pooled frequencies of HLA B51 positivity in adult individuals of sub-Saharan ancestry with Behçet’s disease

Fig. 6

Pooled frequencies of oral (a) and genital (b) ulcers in adult individuals of sub-Saharan ancestry with Behçet’s disease

Fig. 7

Pooled frequencies of skin involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry

Fig. 8

Pooled frequencies of ocular involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry

Fig. 9

Pooled frequencies of central nervous system involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry

Fig. 10

Pooled frequencies of vascular involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry

Fig. 11

Pooled frequencies of joint involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry

Fig. 12

Pooled frequencies of gastro-intestinal involvement related to Behçet’s disease in adult individuals of sub-Saharan ancestry