Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

Gender-Specific Differences in Adamantiades-Behet's Disease Presentation and Their Relationship withHLA-B5: An Analysis of 731 Subjects From the German Registry for Adamantiades-Behet's Disease.

Bonitsis1,   Nikolaos G., Nguyen2,  Liem Binh Luong, Papoutsis1,  Nestor, Altenburg1,  Andreas, Kotter3,  Ina, Mahr2,  Alfred, Zouboulis1,  Christos C.

Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Dessau, Germany
Department of Internal Medicine, Hospital Saint-Louis, Paris, France
Department of Internal Medicine II, Rheumatology Division, University Hospital Tübingen, Tübingen, Germany


Adamantiades-Behçet's disease (ABD) is a multisystem disorder characterized by highly variable presentations. The factors driving the individual disease expressions are not well known. Gender-specific differences in clinical features, such as increased risk of eye involvement in males, have been proposed. The ABD susceptibility allele HLA-B5 is tightly associated with male gender and ocular disease, and it could be hypothesized that the effect of gender on ABD expression is to some extent confounded by HLA-B5 positivity. We undertook a study to investigate the effect of gender on the demographic and clinical features in ABD and to assess whether any differences may account for HLA-B5 status.


This study was based on the German Registry of ABD, a national, multicenter registry prospectively collecting demographic, clinical and genetic information for ABD patients since 1990. Age at diagnosis and cumulative clinical variables (oral ulcers, genital ulcers, pathergy reaction and ocular, skin, joint, neurological, heart, pulmonary, and kidney involvement) were compared accordingly to gender and HLA-B5 status. Statistical analyses used Pearson's chi2 and Student's t tests.


A total of 731 subjects were evaluated (mean age at diagnosis: 27.8 yr [SD 11.7], 58% males, 40% Germans, 44% Turks). The International Study Group classification criteria were satisfied by 629/728 (86%) subjects overall and by 375/423 (89%) males and 254/305 (83%) females (P=0.04). HLA-B5 was found in 350/603 (58%) and was significantly higher in males (relative risk [RR]: 1.27, 95% CI: 1.09–1.48, P=0.001). The age at disease onset did not differ by gender (P=0.22). Male gender was associated with a significantly increased prevalence of ocular involvement (RR: 1.21, 95% CI: 1.06–1.38, P=0.004), skin involvement (RR: 1.11, 95% CI: 1.02–1.21, P=0.01), major vessel involvement (RR: 2.86, 95% CI: 1.89–4.43, P<0.0001) and heart involvement (RR: 9.98, 95% CI: 1.41–203.40, P=0.006) and a decreased prevalence of genital ulcers (RR: 0.<82, 95% CI: 0.74–0.92, P<0.0001) and joint involvement (RR: 0.84, 95% CI: 0.72–0.99, P=0.03). Analyses of the clinical characteristics by HLA-B5 status showed that HLA-B5 was positively associated with ocular disease (RR: 1.43, 95% CI: 1.22–1.67, P<0.0001) and skin involvement (RR: 1.14, 95% CI: 1.04–1.25, P=0.004) and negatively associated with gastrointestinal involvement (RR: 0.51, 95% CI: 0.30–0.86, P=0.008). The HLA status did not affect the links between male gender and eye and skin involvement, respectively, with RR of 1.10 (95% CI: 0.94–1.32, P=0.24) and 1.06 (95 %CI: 0.95–1.19, P=0.34) among HLA-B5 positive patients and 1.15 (95% CI: 0.86–1.55, P=0.35) and 1.18 (95% CI: 1.00–1.39, P=0.05) among HLA-B5 negative patients.


This study adds support to gender-specific differences in ABD clinical presentation and to the link between male gender and HLA-B5 positivity. The fact that HLA-B5 status did not affect the link between gender and clinical presentation suggests that gender and HLA-B5 are independent ABD expression modifiers. These findings may provide further insight into the mechanisms underlying the physiopathology of ABD.

To cite this abstract, please use the following information:
Bonitsis,  Nikolaos G., Nguyen, Liem Binh Luong, Papoutsis, Nestor, Altenburg, Andreas, Kotter, Ina, Mahr, Alfred, et al; Gender-Specific Differences in Adamantiades-Behet's Disease Presentation and Their Relationship withHLA-B5: An Analysis of 731 Subjects From the German Registry for Adamantiades-Behet's Disease. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :2388

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