Background/Purpose: It has been suggested that Behçet’s disease (BD) varies in its phenotypic expression in different ethnies and in different countries indicating that both environmental and genetic factors play a role in the aetiology of the condition. However, most of the evidence supporting these propositions arises from observational case series, which are subject of many sources of bias.
Objective. The present study was undertaken to investigate potential any ethnicity-related differences in the phenotype and prognosis of BD patients in a French multiethnic country.
Methods: We analyzed the phenotype and prognosis of 769 consecutive patients [median (IQR) age at diagnosis of 30.9 (24.9-37.2) years with 535 (69.6%) male] fulfilling the international criteria of classification for BD, in the 3 largest ethnic groups of our cohort [European (n=369), North African (n=350) and sub Saharan African (n=50)].
Results:
Sub Saharan African BD patients had a higher frequency of arterial involvement (28% vs 13.2% vs 10.3%, p=0.004), a higher frequency of CNS involvement (48% vs 32.3% vs 29.5%, p=0 .035), a higher frequency of cardiac involvement (16% vs 7.1% vs 4.3%, p=0.007), a higher rate of death (12% vs 6% vs 3.5%, p=0.029) and a lower frequency of HLA B51 allele (29.4 % vs 49.2% vs 55.8%, p=0.009) compared to those from north Africa and Europe, respectively. The 15-year mortality rate was of 19% in sub Saharan Africa’s BD patients compared to 9% and 6% in those from north Africa and Europe, respectively (p=0.015). Logistic regression analysis showed that male gender (HR: 4.94, CI: 1.53-16.43), and arterial involvement (HR: 2.51, CI: 1.07-5.90) were independently associated with mortality.
Conclusion: In a French multiethnic country, sub Saharan African BD patients exhibited a worse prognosis which is likely related to their vascular phenotype.
Disclosure:
D. Saadoun,
None;
M. Resche Rigon,
None;
B. Wechsler,
None;
D. Le Thi Huong,
None;
J. C. Piette,
None;
P. Cacoub,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/ethnicity-related-differences-in-behcets-disease-in-a-french-multiethnic-country/