title: HLA-B27: an important genetic risk factor for lone aortic regurgitation and severe conduction system abnormalities.
abstract: OBJECTIVE
  HLA-B27, an immunogenetic marker that is present in 8 percent of the white population around the world, has been found to be an important risk factor for the development of a group of rheumatic disorders, the seronegative spondyloarthropathies. Our objective was to assess the possible role of HLA-B27 and the associated inflammatory disease process in the development of lone aortic regurgitation.
  
  
  METHODS
  A group of 91 patients with lone aortic regurgitation were studied by HLA typing and clinical and roentgenologic examination.
  
  
  RESULTS
  The HLA-B27-associated inflammatory disease process was found to be the probable underlying cause in 15 to 20 percent of patients with lone aortic regurgitation of different degrees of severity. Furthermore, HLA-B27 was found in 88 percent of the male patients with the combination of aortic regurgitation and severe conduction system abnormalities.
  
  
  CONCLUSIONS
  We suggest that this cardiac syndrome should be regarded as an HLA-B27-associated syndrome, sometimes part of ankylosing spondylitis or Reiter's disease, but just as often presenting without obvious rheumatic disease. The marker is thus an important and widely distributed risk factor not only for the development of rheumatic disease but also for acquired aortic regurgitation and sever conduction system abnormalities.
authors.affiliation: Department of Medicine, Karolinska Institute, Huddinge Hospital, Stockholm, Sweden.
authors.forename: L
authors.initials: L
authors.lastname: Bergfeldt
authors.forename: P
authors.initials: P
authors.lastname: Insulander
authors.forename: D
authors.initials: D
authors.lastname: Lindblom
authors.forename: E
authors.initials: E
authors.lastname: Möller
authors.forename: O
authors.initials: O
authors.lastname: Edhag
mesh_terms: D001022:Aortic Valve Insufficiency / Q000235:genetics*; D016918:Arthritis, Reactive / Q000235:genetics; D001919:Bradycardia / Q000235:genetics; D005260:Female; D006680:HLA Antigens / Q000235:genetics*; D015796:HLA-B27 Antigen; D006329:Heart Conduction System / Q000503:physiopathology*; D006650:Histocompatibility Testing; D006801:Humans; D008297:Male; D012216:Rheumatic Diseases / Q000235:genetics*; D012307:Risk Factors; D013167:Spondylitis, Ankylosing / Q000235:genetics
chemical_list: D006680:HLA Antigens; D015796:HLA-B27 Antigen