Background Behcet’s disease (BD) is characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, or arthritis. Increased risk of developing BD is associated with the presence of certain human leukocyte antigens (HLA), particularly HLA-B51. However prevalence of B51 is only half of BD1). Recently, A26 and B52 have been also reported to be associated with BD, still those HLA have been not detected by many patients2). Ankylosing spondylitis(AS) is associated with HLA-B27. HLA B7, Bw22(B54,55,56), B39, B40(B60), B61, B67 and B73 have been reported the amino acid sequence homology and the antigenic homology with HLA-B273) and called HLA-B27-associatedHLA-factor. Uveitis, arthritis, and oral ulcers are common symptoms of AS and BD.
Objectives To investigate whether HLA-B27-associated HLA factors are newer genetic factors related to BD.
Methods We retrospectively reviewed 55 BD patients who performed HLA typing at our institutes from 2006 to 2011. They were diagnosed according to the 1990 criteria for BD. Patients with sacroiliitis and/or spondylitis were excluded. Patient with psoriasis, Reiter’s syndrome and inflammatory bowel diseases were excluded similarly. All patients were assessed by a rheumatologist. HLA-A and -B typing were done by a PCR-SSOP(reverse sequence specific oligonucleotide) -Lumines method. Control group: a total of 159696 healthy donors(male:female ratio 1.6:1). Their HLA data were obtained from the Japanese Red Cross Society4). All cases and controls were of Japanese.
Results Among the 55 BD patients, male to famale ratio was 19:36. Thirteen patients were HLA-B51-positive (23.6%), 10 were HLA-B52-positive (18.2%) and 7 were HLA-A26-positive (12.7%) (Table 1). In the remaining 21 patients with BD, 17 patients (81%) had HLA-B27-associated HLA factor (Table 2). Only four patients (4/55, 7.3%) were negative for all HLAs mentioned before. The frequency of HLA-B39and B54was significantly higher in BD patients. Only four patients (7.3%) were negative of either factor (HLA-B51,B52, A26 and B27-associated HLA factor). 58% of HLA-B27-associated HLA factor-positive patients had arthritis without spondylitis.
Conclusions Most BD patients are related to HLA-B51,52, A26 and B27-associated HLA factor. The HLA-B27-associated HLA factor is possible of one of genetic component in BD patients.
Behçet’s disease. Sakane T et al. N Engl J Med. 1999;341:1284.
Close association of HLA-B51 and B52 in Israeli patients with Behçet’s syndrome. Arber N et al. Ann Rheum Dis. 1991;50:351.
Anti-HLA-B7, B27, Bw42, Bw54, Bw55, Bw56, Bw67, Bw73 monoclonal antibodies: specificity, idiotypes, and application for a double determinant immunoassay. Sakaguchi K et al. Hum Immunol. 1988 Mar;21:193-207.
Analysis of HLA gene frequencies and HLA haplotype frequencies for bone marrow doners in Japan. Moriyama Y et al. MHC 2006;12:25-43
Disclosure of Interest None Declared