Abdominal aortitis in HLA-B27+ spondyloarthritis: Case report with 5-year follow-up and literature review

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Abstract

Objectives

Aortic disease is a known complication of HLA-B27-associated spondyloarthritis. We present the case of a 52-year-old HLA-B27-positive woman with aortitis of the abdominal aorta and spondyloarthritis.

Methods

In addition to the case reported, a literature search (MEDLINE) for articles published between 1946 and September 2013 was performed using combinations of the MEDLINE subject headings keywords “spondylarthritis,” “ankylosing spondylitis,” “reactive arthritis,” “psoriatic arthritis,” “aortitis,” and “abdominal aorta.” Relevant references were retrieved.

Results

Case report: Our patient presented to the ER in June 2008 with a 3-week history of worsening of severe cramping lower abdominal pain. Her history also included recurrent acute episodes of iritis, which eventually led to enucleation of her left eye despite treatment with corticosteroids. CT of the abdomen showed findings suggesting aortitis of the abdominal aorta. She responded to therapy with prednisone, and follow-up imaging showed eventual resolution of the aortitis. She later went on to be diagnosed with psoriatic arthritis with spondylitis.

Review of literature: Six previously reported cases of abdominal aortitis in spondyloarthritis were found. Four of these were reported in patients with ankylosing spondylitis, one in reactive arthritis, and one in psoriatic arthritis. The first case reported was in 1958 and the most recent in 2012.

Conclusions

Rheumatologists should be aware of the possibility of abdominal aortitis occurring in their patients with SpA and should consider it as part of the differential diagnosis in a SpA patient with unexplained visceral pain or systemic features out of proportion to clinically apparent disease.

Introduction

Heart disease is a known complication of HLA-B27-associated spondyloarthritis (SpA). The common lesions are aortic regurgitation and conduction defects. Older autopsy studies show that 10–20% of patients with ankylosing spondylitis (AS) have evidence of aortitis, usually not clinically recognized [1]. Typically, the ascending aorta is affected. In 2008, we encountered a 52-year-old First Nations woman with aortitis of the abdominal aorta presenting with intractable abdominal pain. A review of the literature uncovered six previously described cases of aortitis of the abdominal aorta associated with SpA. Herein, we describe our patient׳s clinical presentation, treatment, 5-year follow-up, and a review of previously reported cases.

Section snippets

Case report

A 52-year-old woman from the Taku River Tlingit First Nation, Atlin, near Yukon, presented to the St. Paul׳s Hospital emergency room in June 2008 with a 3-week history of worsening of severe cramping lower abdominal pain interfering with sleep and unresponsive to analgesics. She reported a past 2-year history of similar intermittent epigastric and suprapubic cramps of various descriptions, leading to hospitalization in the North. Investigations had been performed for suspected cholecystitis,

Methods

A literature search (MEDLINE) for articles published between 1946 and September 2013 was performed using combinations of the MEDLINE subheadings keywords “spondylarthritis,” “ankylosing spondylitis,” “reactive arthritis,” “psoriatic arthritis,” “aortitis,” and “abdominal aorta.” Relevant references were retrieved. Additional relevant articles were found by checking the references quoted within the retrieved articles.

Review of literature

Six cases of abdominal aortitis have been previously reported in SpA. Four of these cases were reported in patients with AS, one in reactive arthritis, and one in psoriatic arthritis [2], [3], [4], [5], [6], [7].

The first case of abdominal aortitis in SpA was reported by Ansell et al. [2] in 1958. The patient was a 27-year-old male with AS and chronic iritis, leading to vision loss. He suffered from angina, congestive heart failure, and died from pulmonary embolism. On autopsy, the aorta showed

Discussion

Aortic regurgitation and aortitis of the ascending aorta are well-known complications of AS. Abdominal aortitis is rare. Our case is the seventh reported case of abdominal aortitis in SpA. All of the reported cases are in patients with severe AS, psoriatic arthritis, and reactive arthritis. Three of the seven, including our own case, had severe vision-threatening iritis [2], [3]. Two of seven patients were on established anti-TNF therapy, leading to speculation by the case report authors

Conclusion

Rheumatologists should be aware of the possibility of abdominal aortitis occurring in their patients with SpA, and should consider it as part of the differential diagnosis in a SpA patient with unexplained visceral pain or systemic features out of proportion to clinically apparent disease.

Acknowledgment

We thank Wendy Lin for her work on this project in the summer of 2010.

References (7)

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    A recent study of 187 patients with AS investigated by transthoracic echocardiography showed evidence of aortic insufficiency in 34 patients, mild in 24, moderate in 9 and severe in 1, suggesting that the subclinical aortic valve disease which, as mentioned above, is at least in part a consequence of aortitis, may still be significantly overrepresented in these patients [48]. There are a number of case reports of different types of vasculitis that appear in the literature, including cutaneous vasculitis in ankylosing spondylitis, thoraco-abdominal aortitis in ankylosing spondylitis [49], polyarteritis nodosa in psoriatic arthritis in a child, retinal vasculitis in ankylosing spondylitis as well as cerebral vasculitis, and cutaneous Polyarteritis in Reiter's disease and ankylosing spondylitis. The relevance of these is very difficult to interpret because they appear in isolation and may be unrelated.

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Role of the funding source: This project was completed with support from the Canadian Rheumatology Association Summer Studentship Program. The sponsors had no involvement in this study.

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