HLA-B27 and the seronegative spondyloarthropathies

Am J Med Sci. 1998 Oct;316(4):239-49. doi: 10.1097/00000441-199810000-00004.

Abstract

In the 25 years since the initial reports of the association of HLA-B27 with ankylosing spondylitis (AS) and subsequently with Reiter's syndrome, psoriatic spondylitis, and the spondylitis of inflammatory bowel disease, the association of HLA-B27 with the seronegative spondyloarthropathies has remained one of the best examples of a disease association with a hereditary marker. HLA-B27 has been recognized as representative of a spectrum of diseases, ranging from the majority of HLA-B27-positive individuals who have no disease at all, through those with isolated eye or skin involvement, to those with critical eye, heart, and peripheral joint compromise of full-blown AS. Yet HLA polymorphism has evolved in response to environmental stresses, and even the presence of HLA-B27 itself appears to confer advantages in certain infectious diseases, such as acquired immune deficiency syndrome (AIDS). This article will review what is currently known about HLA-B27 and disease, especially in the seronegative spondyloarthropathies. The structure-function relationship of HLA-B27 will be presented, including differences between the B27 subtypes both in their ethnic variation and possible disease implications. The disease spectrum conferred by the presence of HLA-B27 will also be discussed, and the theories of how HLA-B27 contributes to the pathogenesis of the spondyloarthropathies will be considered.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Arthritis, Reactive / blood
  • Arthritis, Reactive / immunology*
  • Global Health
  • HLA-B27 Antigen / blood*
  • HLA-B27 Antigen / chemistry
  • HLA-B27 Antigen / genetics
  • Humans
  • Inflammatory Bowel Diseases / blood
  • Inflammatory Bowel Diseases / immunology
  • Models, Molecular
  • Molecular Sequence Data
  • Protein Structure, Secondary
  • Psoriasis / blood
  • Psoriasis / immunology
  • Sequence Alignment
  • Spondylitis / blood
  • Spondylitis / immunology*
  • Spondylitis, Ankylosing / blood
  • Spondylitis, Ankylosing / immunology*

Substances

  • HLA-B27 Antigen