title: Behçet's disease in East African patients may not be unusual and is an HLA-B51 negative condition: a case series from Mayotte (Comoros).
abstract: 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.
authors.affiliation: Medicine Department, Mayotte's Hospital, Mamoudzou, Mayotte.
authors.forename: Eric
authors.initials: E
authors.lastname: Liozon
authors.forename: Céline
authors.initials: C
authors.lastname: Roussin
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.forename: Alain
authors.initials: A
authors.lastname: Garou
authors.forename: Philippe
authors.initials: P
authors.lastname: Valadier
authors.forename: Ian
authors.initials: I
authors.lastname: Périnet
authors.forename: Loïc
authors.initials: L
authors.lastname: Raffray
authors.forename: Yves
authors.initials: Y
authors.lastname: Théry
authors.forename: Bruno
authors.initials: B
authors.lastname: De Lagarde
mesh_terms: D000328:Adult; D000351:Africa, Eastern / Q000453:epidemiology; D001528:Behcet Syndrome / Q000188:drug therapy / Q000453:epidemiology* / Q000276:immunology*; D016022:Case-Control Studies; D003078:Colchicine / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D015235:HLA-B Antigens / Q000378:metabolism*; D059949:HLA-B51 Antigen; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D016896:Treatment Outcome; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D015235:HLA-B Antigens; D059949:HLA-B51 Antigen; D007166:Immunosuppressive Agents; D050257:Tubulin Modulators; D003078:Colchicine