HLA-B27 positive juvenile arthritis with cardiac involvement preceding sacroiliac joint changes

Abstract

While cardiovascular disease develops in up to 50% of adult patients with ankylosing spondylitis, it is very uncommon in its juvenile counterpart. Regarding the early stage of the disease, before onset of sacroiliac joint changes, only two cases with aortic incompetence have been published while reports of mitral valve involvement are not available. A 13 year old boy is described with an HLA-B27 positive asymmetric oligoarthritis and enthesitis, without back pain or radiographic evidence of sacroiliitis. Echocardiography showed an echogenic structure measuring 8 x 11 x 20 mm at the fibrous continuity between the aortic and mitral valves extending through a false tendon into an echogenic thickened posterior papillary muscle, causing a grade II aortic and grade I/II mitral regurgitation. Short term corticosteroid and long term non-steroidal anti-inflammatory drug and disease modifying antirheumatic drug treatments efficiently controlled the symptoms. The cardiac findings remained unchanged during a follow up of 20 months. Careful cardiac evaluation appears to be mandatory even in these young patients.

Figure 1

Echogenic 8 × 11 × 20 mm structure between the aortic and mitral valves (arrows, parasternal long axis (upper left), parasternal short axis (lower left)) connected by a false tendon to the echogenic posterior papillary muscle (arrows, parasternal long axis (upper right), parasternal short axis (lower right)).

Figure 2

Colour Doppler echocardiography showing valve dysfunction: grade II aortic regurgitation (arrows, parasternal long axis (left)) and grade I/II mitral regurgitation (arrows, parasternal long axis (right)).