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[Horton's disease and aortic aneurysm: coincidence or causality? 5 cases]
J M Cormier1, F Cormier, D Laridon
1Services de Chirurgie Vasculaire, Clinique de la Défense, 16, Bd Emile Zola, 92000 Nanterre.
Insights
Giant cell arteritis can lead to inflammatory aortopathies, including aortic aneurysms and dissections, years later. Early detection and monitoring of patients with giant cell arteritis are crucial for preventing life-threatening aortic complications.
Area of Science:
- Vascular Surgery
- Rheumatology
- Cardiology
Context:
- Giant cell arteritis (GCA) is a systemic vasculitis primarily affecting medium and large arteries.
- Aortic involvement in GCA, including aneurysms and dissections, can occur years after initial diagnosis.
- The association between GCA and inflammatory aortopathies is increasingly recognized.
Purpose:
- To report five cases of inflammatory aortopathies occurring after giant cell arteritis diagnosis.
- To highlight the clinical presentation, diagnostic challenges, and management of these cases.
- To emphasize the importance of vigilant surveillance for aortic complications in GCA patients.
Summary:
- Five patients developed inflammatory aortopathies (aneurysms, dissections) 3-16 years post-GCA diagnosis.
- Symptomatic cases presented with severe inflammatory syndrome; asymptomatic cases were found during follow-up.
- Corticosteroid therapy was effective in managing the inflammatory process, and surgical intervention was required for two aneurysms.
Impact:
- This study underscores that aortic aneurysms and dissections in patients with GCA are not coincidental but represent a significant association.
- It suggests that GCA should be considered in younger patients (<50) presenting with inflammatory aortopathies.
- Recommends annual thoracic radiography and regular ultrasound/Doppler examinations for GCA patients to detect aortic involvement early, preventing fatal outcomes like rupture or dissection.
Abstract:
Five inflammatory aortopathies were disclosed 3 to 16 years after inaugural giant cell arteritis. Three patients were symptomatic: one aneurysm of the subrenal abdominal aorta discovered at work-up for an inferior arteriopathy, one thoraco-abdominal aneurysm with a "fissuration" episode, one calcified thoraco-abdominal aortopathy suggesting dissection. In these three cases, there was a severe inflammatory syndrome with asthenia, fever, elevated erythrocyte sedimentation rate and a large inflammatory crown around the aortopathy. In the two asymptomatic cases, the diagnosis was made during the follow-up of Horton's disease, in one patient with active disease, the other late after the initial episode. Two aneurysms required surgical cure, with resection-prosthesis of the thoraco-abdominal aneurysm and revascularization of the digestive and renal arteries. In the 4 active cases, corticosteroid therapy cured the inflammatory process both on the basis of laboratory results and the involution of the periaortic crown and, in one case, the total regression of ureteral compression causing pyeloureteral dilatation on the left. The diagnosis of giant cell arteritis was confirmed histologically in the two operated cases. Extra-cervical localizations of aortic aneurysm of dissection in patients with giant cell arteritis is not a fortuitous coincidence but an association as demonstrated by the Mayo Clinic epidemiology. On the basis of these reported cases and data in the literature, the practical conclusions are: in case of aorta involvement, particularly with inflammation in subjects under 50, giant cell arteritis should be entertained as a possible diagnosis; in patients with giant cell arteritis, follow-up should include yearly thoracic radiograms to search for thoracic aorta involvement and Doppler and ultrasound explorations to identify any abdomino-iliac lesions. This protocol is required to avoid the life-threatening complications of dissection or rupture of an aortic aneurysm.