title: Gender-specific differences in Adamantiades-Behçet's disease manifestations: an analysis of the German registry and meta-analysis of data from the literature.
abstract: OBJECTIVE
  We investigated the effect of gender on the clinical Adamantiades-Behçet's disease (ABD) phenotype with data from the German ABD registry and a meta-analysis from a systematic literature review.
  
  
  METHODS
  Using the German ABD registry data, we compared 36 clinical variables by gender (with women as the reference category) and investigated potential effect modification by HLA-B5 or ethnic background. The registry data were combined with those from a literature search to calculate pooled relative risks (RRs) for variables with data from ≥10 relevant datasets.
  
  
  RESULTS
  The German ABD registry provided information for 747 subjects (58.1% males) and the systematic literature review identified another 52 datasets informing on 16 variables. Both analyses consistently revealed the association of male gender with ocular involvement (RR 1.28 and 1.34 from the ABD registry and meta-analysis, respectively), folliculitis (RR 1.30 and 1.26), papulopustular lesions (RR 1.23 and 1.25), vascular involvement (RR 2.31 and 2.27), superficial (RR 2.96 and 1.63) and deep venous thromboses (RR 2.56 and 2.16) and female gender with genital ulcers (RR 0.78 and 0.92) and joint involvement (RR 0.79 and 0.89). The ABD registry data additionally showed male gender associated with heart involvement (RR 10.60), whereas the meta-analyses revealed male gender associated with the pathergy test (RR 1.14) and female gender associated with erythema nodosum (RR 0.86). HLA-B5 and Turkish or German origin did not affect the observed associations.
  
  
  CONCLUSIONS
  These analyses support gender-associated clinical variations in ABD and in particular a clinically meaningful risk of cardiovascular involvement for men.
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France. Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Nikolaos G
authors.initials: NG
authors.lastname: Bonitsis
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Liem B
authors.initials: LB
authors.lastname: Luong Nguyen
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Michael P
authors.initials: MP
authors.lastname: LaValley
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Nestor
authors.initials: N
authors.lastname: Papoutsis
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France. Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Andreas
authors.initials: A
authors.lastname: Altenburg
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Ina
authors.initials: I
authors.lastname: Kötter
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Christiana
authors.initials: C
authors.lastname: Micheli
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Carla
authors.initials: C
authors.lastname: Maldini
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France. Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France.
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.affiliation: Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France. Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, German Registry of Adamantiades-Behçet's Disease, Dessau, Germany, Department of Internal Medicine, Hospital Saint-Louis, Paris, France, Department for Statistics, School of Public Health, Boston University, Boston, MA, USA and ECSTRA Team, Epidemiology and Biostatistics, Sorbonne Paris Cité Research Center UMR 1153, Inserm, University Paris Diderot, Paris, France. alfred.mahr@sls.aphp.fr.
authors.forename: Christos C
authors.initials: CC
authors.lastname: Zouboulis
mesh_terms: D000293:Adolescent; D000328:Adult; D001528:Behcet Syndrome / Q000150:complications* / Q000276:immunology / Q000473:pathology*; D005260:Female; D005499:Folliculitis / Q000453:epidemiology / Q000209:etiology; D005831:Genital Diseases, Female / Q000453:epidemiology / Q000209:etiology; D005858:Germany; D015235:HLA-B Antigens / Q000097:blood; D006801:Humans; D015994:Incidence; D007592:Joint Diseases / Q000453:epidemiology; D008297:Male; D010641:Phenotype; D012042:Registries; D012307:Risk Factors; D012737:Sex Factors; D020246:Venous Thrombosis / Q000453:epidemiology / Q000209:etiology; D055815:Young Adult
chemical_list: D015235:HLA-B Antigens; C035270:HLA-B5 antigen
keywords: Adamantiades-Behçet’s disease; Behçet’s disease; Behçet’s syndrome; gender; meta-analysis; sex factors