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HLA-B27-Associated Cardiac Disease

Lennart Bergfeldt, MD, PhD
[+] Article and Author Information

From Karolinska Institute and Hospital, Stockholm, Sweden; and Stanford University School of Medicine, Stanford, California. For the current author address, see end of text. Grant Support: In part by the Swedish Medical Research Council, the Karolinska Institute and Hospital, the Swedish Medical Society, the Swedish Heart-Lung Foundation, and the Seraphimer Foundation. Requests for Reprints: Lennart Bergfeldt, MD, PhD, Department of Cardiology, Karolinska Hospital, PO Box 110, S-171 76 Stockholm, Sweden.


Copyright ©2004 by the American College of Physicians


Ann Intern Med. 1997;127(8_Part_1):621-629. doi:10.7326/0003-4819-127-8_Part_1-199710150-00007
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Purpose: To review the available information about cardiac disease in relation to the immunogenetic marker HLA-B27 and the inflammatory disorders associated with it (seronegative spondyloarthropathies).

Data Sources: English-language clinical studies, case reports, and reviews published in 1995 or earlier were identified from a search of the MEDLINE database. These papers, textbooks, and other bibliographic sources were used to identify other relevant publications, enabling collection of references from 1936 through 1995 in a stepwise manner.

Study Selection: Clinical studies, case reports, and topical reviews on the HLA-B27 histocompatibility locus, seronegative spondyloarthropathies, and cardiac disease were selected.

Data Extraction: Information from systematic or hypothesis-driven studies, including those showing clinically and statistically meaningful associations between disorders, and additional relevant information from case reports and reviews is presented.

Data Synthesis: Immunogenetic, histopathologic, clinical, and electrophysiologic evidence was examined to explore the concept of HLA-B27-associated cardiac disease.

Results: Relative to the general population (in which the frequency is 6% to 8%), men (but not women) with pace-makers have a significantly increased frequency of HLA-B27. Furthermore, a cardiac syndrome that consists of severe conduction system abnormalities plus aortic regurgitation is associated with HLA-B27, which was present in 67% to 88% of the patients with both of these clinical findings. This proportion of HLA-B27 is similar to that found in patients with ankylosing spondylitis and Reiter disease. Cardiac and aortic tissue both show intimal proliferation of small arteries (obliterative endarteritis) and fibrosis, as seen in tissues adjacent to afflicted joints. Both cardiac conduction abnormalities and aortic regurgitation occur in patients with various HLA-B27-related extracardiac disorders, regardless of the severity of the latter; in about 50% of patients with these cardiac findings, a diagnosis of an HLA-B27-related rheumatic disease has not previously been made. Third-degree (complete) atrioventricular block related to HLA-B27 is located within the atrioventricular node in 95% rather than the expected 20% of cases.

Conclusions: The heart and the joints are both of major importance as targets for the HLA-B27-associated disease process. HLA-B27-related cardiac lesions may be found in the absence of other rheumatologic manifestations. Furthermore, a genetically linked cardiac syndrome has been defined: the combination of conduction system abnormalities and aortic regurgitation.

Figures

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Figure 1.
Aortic valve from a patient with ankylosing spondylitis.[12]

The valve and sinus of Valsalva are cut open. The aortic valve (circumference, 80 mm) and the ascending aorta are both dilated. The base of the aortic cusps is thickened, and the free margins are rolled. All three features contribute to aortic valve insufficiency. The arrow indicates the subaortic ridge caused by fibrosis extending downward from the nonseptal portion of the aorta onto the anterior mitral leaflet. This can impair the mobility of the anterior mitral leaflet and contribute to mitral valve insufficiency. Reproduced from Bergfeldt and colleagues with permission.

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Figure 2.
Intimal proliferation within the atrioventricular nodal artery of a patient with HLA-B27-associated cardiac disease.

(Courtesy of Professor Michael J. Davies, St. George's Hospital, London.).

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