HLA-B27-associated cardiac disease

Area of Science:

• Immunogenetics
• Cardiology
• Rheumatology

Background:

• The human leukocyte antigen B27 (HLA-B27) is an immunogenetic marker associated with inflammatory disorders.
• Seronegative spondyloarthropathies are inflammatory conditions linked to HLA-B27, often affecting the joints.
• Previous research suggested a potential connection between HLA-B27 and cardiac involvement.

Purpose of the Study:

• To review existing literature on cardiac disease in relation to the HLA-B27 marker.
• To explore the association between HLA-B27 and inflammatory disorders like seronegative spondyloarthropathies.
• To investigate the characteristics of HLA-B27-associated cardiac conditions.

Main Methods:

• A comprehensive literature search was conducted using MEDLINE and other bibliographic sources.
• English-language clinical studies, case reports, and reviews published up to 1995 were included.
• Data extraction focused on studies examining HLA-B27, seronegative spondyloarthropathies, and cardiac disease.

Main Results:

• Men with pacemakers showed a significantly higher frequency of HLA-B27 compared to the general population.
• A distinct cardiac syndrome, characterized by severe conduction abnormalities and aortic regurgitation, was strongly associated with HLA-B27 (67%-88% of cases).
• Histopathological findings in cardiac and aortic tissue revealed intimal proliferation and fibrosis, similar to changes seen in affected joints.

Conclusions:

• The heart and joints are key targets of the HLA-B27-associated disease process.
• Cardiac lesions related to HLA-B27 can manifest independently of other rheumatologic symptoms.
• A genetically defined cardiac syndrome comprising conduction abnormalities and aortic regurgitation has been identified.