Valvular heart disease associated with juvenile onset ankylosing spondylitis: a case report and review of the literature.

Aortitis with associated aortic insufficiency is a well-known extra-articular manifestation of ankylosing spondylitis and other HLA-B27 associated spondyloarthropathies in adults. The cardiovascular histopathology is distinct from other forms of inflammatory and degenerative valve disease. Clinical cardiac involvement may be slowly progressive or fulminant and life-threatening. Mitral valve involvement associated with juvenile onset ankylosing spondylitis has rarely been reported. This article discusses a case of juvenile onset spondylitis in which associated aortic, and possibly mitral, valve disease dominated the clinical course for many years before axial joint disease required total replacement of the right hip.