Behçet's disease in East African patients may not be unusual and is an HLA-B51 negative condition: A case series from Mayotte (Comoros)

Objective: We aimed to report the presence of Behçet's disease in patients of East African ancestry, where there have been very few reports of the disease. Methods: Case series of 14 patients, all of whom were born in the Comoros, reporting to the single primary general medicine department of Mayotte's island since 1998. All the patients but one satisfied the international group study criteria for Behçet disease. Results: We report a series of 14 patients (13 unrelated) including 10 men and features of the disease. Two multicase family histories were elicited. Mean diagnosis delay was 5.5. ± 5.1. years. Behçet's disease presented mainly as neuro-Behçet in five patients including three with progressive brain stem syndrome, vascular disease in three patients, relapsing panuveitis in two patients, and rheumatic disease with mucocutaneous disease in four patients. Thirteen patients were tested and were found to be HLA-B51 negative. All the patients were treated with colchicine and most of them received additional immunosuppressive treatment, mainly glucocorticoids and azathioprine. However, after a mean 43-month follow-up, five had serious permanent disabling, one of whom had died of neuro-Behçet. Conclusions: Behçet's disease may be an under-reported, HLA-B51 negative, condition in native East African populations and appears to be often life-threatening and/or associated with severe damage in these patients. A high degree of awareness of physicians is necessary to shorten diagnostic delays and to improve the management of patients. © 2010 Société française de rhumatologie.