Aortitis and spondyloarthritis--an unusual presentation: case report and review of the literature

Objectives: To report a patient with aortic regurgitation (AR) and aortitis, who failed to respond to multiple attempts at surgical replacement of the affected valve and adjacent aorta, and in whom a late diagnosis of spondyloarthritis (SpA) was made. The relevant literature on the association of SpA and aortitis is reviewed.

Methods: Descriptive case report of a patient with AR secondary to aortic dilation and aortitis with a late diagnosis of SpA, and a review of the relevant literature (PubMed search 1956 through April 2008).

Results: A 36-year-old man was admitted to the hospital for replacement of his aortic valve and aortic valve root as a result of AR after a dehiscence of a mechanical prosthetic valve and subsequent aortic pseudoaneurysm. His past medical history included 2 prior aortic valve replacements that failed due to late postoperative dehiscence. Histological findings from the aortic wall after the third operation showed signs of aortitis. A rheumatologic workup revealed a 6-year history of mild low back pain, radiological evidence of bilateral sacroiliitis, and presence of HLA-B27. A diagnosis of SpA, most likely ankylosing spondylitis (AS), was made. On reviewing the literature, AR has been diagnosed in 2 to 12% of the patients with AS, most of whom had long-lasting disease. There have been several reports of uncommon presentations of acute AR in young patients with juvenile AS; however, no similar adult case was found.

Conclusion: This unusual case emphasizes the importance of early and adequate workup for SpA in young patients with lone AR or aortic dilation.