Long-term follow-up of aortic involvement in giant cell arteritis: a series of 48 patients

Abstract

To date, only a few series have analyzed the long-term outcome of giant cell arteritis (GCA) patients with aortic involvement, which prompted us to conduct the current retrospective study. Our aims were to 1) determine the prevalence of GCA in patients exhibiting nonatherosclerotic aortic involvement (that is, aortitis, aortic ectasia, and/or aneurysm); and 2) evaluate clinical features and long-term outcome of GCA patients exhibiting aortitis, aortic ectasia, and/or aortic aneurysm.From January 1997 to March 2008, 66 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of nonatheromatous aortic complications (aortitis, aortic ectasia, and/or aneurysm). In these 66 patients, aortic involvement was related to GCA (n = 48), Takayasu arteritis (n = 6), relapsing polychondritis (n = 1), and infection (n = 11).Of the 48 patients with GCA, aortic involvement preceded the initial GCA diagnosis in 1 patient. Aortic involvement was identified in association with GCA in 40 patients (83.3%), and developed after the onset of GCA in the 7 remaining patients (14.6%). Aortic involvement was more often asymptomatic (77.1%). The aortic helical computed tomography (CT)-scan procedure principally showed isolated aortitis (circumferential thickening of the aortic wall >3 mm) in 41 patients (85.4%). In the remaining 7 patients with GCA (14.6%), aortic helical CT scan demonstrated aortic thoracic ectasia and aortitis (n = 3), aortic thoracic aneurysm and both thoracic and abdominal aortitis (n = 3), and both aortic abdominal aneurysm and aortitis (n = 1). All patients were given steroid therapy at a median daily dose of 1 mg/kg initially.At 6-month follow-up, 34 of 48 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan demonstrated complete disappearance of aortitis in 8.8% of patients, improvement of aortitis in 47.1%, unchanged pattern of aortitis and/or aortic thoracic ectasia/aneurysm in 41.2%, and deterioration of aortic thoracic aneurysm in 1 patient (2.9%). At 18-month follow-up, 11 patients systematically underwent both thoracic and abdominal CT scan. Aortic helical CT scan showed complete disappearance of aortitis (n = 1), improvement of aortitis (n = 1), unchanged pattern of aortic thoracic ectasia/aneurysm (n = 2), and deterioration of aortic thoracic aneurysm (n = 1). At patients' last follow-up, the median daily dose of prednisone was 7 mg. Steroid therapy could be discontinued in 17 patients (35.4%).The current retrospective study suggests that aortic impairment may be more prevalent than previously reported. Our findings suggest that specific inflammatory thickening of the aortic wall is common at the time of GCA diagnosis, and that aortitis may be the first manifestation of GCA-associated aortic complications. Whether isolated aortitis leads to vascular wall injury responsible for late-onset aneurysmal disease remains to be determined. At this time, we recommend long-term monitoring for aortic aneurysms, especially in high-risk subjects, although the optimal frequency and imaging modality have not yet been determined. A yearly screening strategy for thoracic/abdominal aortic aneurysms has been proposed for patients with GCA, including physical examination, 2-view chest radiograph, and abdominal ultrasound.