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Case
A 53-year-old woman with a history of ventricular ectopic ablation was admitted with weight loss, sweats and low grade fever and was found to have severe aortic incompetence and a dilated left ventricular cavity (6.1 cm) on transthoracic echocardiography (figure 1). No vegetations were seen on transoesophageal echocardiography and sequential blood cultures were negative. Despite extensive investigation she continued to deteriorate clinically and had a persistently elevated C reactive protein and erythrocyte sedimentation rate at 300 mg/l and 108 mm/h respectively. A positron emission tomography (PET)-CT scan with 18F-fluorodeoxyglucose (FDG) showed a striking increase in FDG uptake throughout the whole aorta as well as the subclavian and iliac arteries (figure 2), confirming a large vessel arteritis. She was treated with high-dose methylprednisolone for three days with a dramatic response, followed by an oral tapering regime. The patient remained well under routine outpatient follow-up with both the Cardiology and Rheumatology specialist teams.
Aortitis is an inflammatory process of one or more layers of the aortic wall with large vessel arteritis, the most common cause of non-infective aortitis. The presence of aortic incompetence is associated with a poorer prognosis.1 Multi-modality imaging is recommended while FDG-PET/CT has a role in the diagnosis of large vessel vasculitis affecting the aorta though caution must be exercised, as FDG-PET/CT will detect just over half of affected patients.2 Management consists of immunosuppression with high-dose corticosteroid therapy. Surgery and percutaneous intervention are best avoided due to the early complications associated with aortic wall fragility, prosthetic valve dehiscence and refractory inflammation despite optimal anti-inflammatory therapy.1
Acknowledgments
Dr Patrick Kiely, (Consultant Rheumatologist, St George's Healthcare NHS Trust) for his help with patient care.
Footnotes
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Contributors MSR: patient care, prepared manuscript and images. NS: patient care, literature search. LJA: in charge of patient care, final editing of manuscript.
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Competing interests None.
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Patient consent Obtained.
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Ethics approval Informed written consent was obtained from the patient and submitted with the manuscript.
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Provenance and peer review Not commissioned; internally peer reviewed.