Abdominal aortitis in HLA-B27+ spondyloarthritis: Case report with 5-year follow-up and literature review
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.
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Cited by (8)
Comorbidities
2019, Axial SpondyloarthritisCardiovascular Involvement in Ankylosing Spondylitis
2017, Handbook of Systemic Autoimmune DiseasesCitation Excerpt :The histological pattern of aortitis in AS is thus lymphoplasmacytic (Stone et al., 2015). Although rare, thoracic or abdominal aorta may also be affected (Hull et al., 1984; Grewal et al., 2014). One of the most important consequences of aortic involvement is the development of “lone” AI, which refers to aortic incompetency without accompanying stenosis (Bergfeldt, 1997).
Vasculitis and inflammatory arthritis
2016, Best Practice and Research: Clinical RheumatologyCitation Excerpt :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.
Aortitis due to giant cell arteritis and psoriatic arthritis: An uncommon association
2017, Reumatologia ClinicaClinical Manifestations, Differential Diagnosis, and Laboratory Markers
2021, Rare Diseases of the Immune System
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.