Aortic insufficiency in a patient with reactive arthritis: case report and review of the literature.

Abstract

Reactive arthritis (ReA) consists of the classic clinical triad of arthritis, urethritis, and conjunctivitis generally occurring within 6 weeks of an infection, typically of the gastrointestinal or genitourinary systems. Cardiovascular manifestations of ReA and other members of the spondyloarthritis family have long been recognized. A 43-year-old male who was human leukocyte antigen-27 (HLA-B27)-positive and who had ReA for 19 years developed severe aortic insufficiency requiring aortic valve replacement. Typically, the onset of musculoskeletal symptoms precedes development of aortic insufficiency by many years. The average calculated from reported cases was 13 years, with a range from 4 days to 61 years. The mechanism by which the aortic valve leaflets become targets in HLA-B27-associated disease is unclear. At one point, interest developed as to whether the HLA-B27 allele was independently associated with lone aortic insufficiency, in the absence of clinical spondylitis. The preponderance of cardiac abnormalities in patients with HLA-B27-positive ReA has led to the suggestion that a genetic syndrome of the heart consisting of aortic insufficiency and conduction-system abnormalities exists, and has been dubbed the "HLA-B27-associated cardiac syndrome". This case highlights the importance of recognizing the association between HLA-B27-associated spondyloarthritis and serious aortic valvular complications. Clinicians should maintain a high suspicion for aortic insufficiency in patients with ReA, including a low threshold for echocardiographic evaluation. A heightened awareness can lead to earlier identification and potential avoidance of fatal events in these patients.