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Published on: March 28, 2025
Acute dissecting thoracic aortic aneurysm in a patient with polymyalgia rheumatica
Tomohiro Koga1, Taichiro Miyashita, Yojiro Matsuoka
1Department of General Internal Medicine, NHO National Nagasaki Medical Center, Nagasaki, Japan.
Abstract:
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related disorders found in older patients, and vasculitis has been proposed as a part of the pathogenesis of PMR. We describe a female patient with PMR plus aortitis, both of which were well controlled on maintenance steroid therapy. Six months after the onset of her condition, however, she suddenly presented with chest pain. A diagnosis of dissecting aortic aneurysm was confirmed, and the aorta was successfully resected. Histology revealed infiltration of mononuclear cells including giant cells around the vaso vasorum with disruption of elastic lamina of the resected aorta. PMR or GCA may indicate an increased risk for aortic dissection in patients with normal erythrocyte sedimentation rate or C-reactive protein, and prompt recognition and therapy, not only during the active disease but also after symptoms of PMR have resolved, are needed.
Insights
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) can increase aortic dissection risk. Early recognition and treatment are crucial, even after initial symptoms resolve.
Area of Science:
- Rheumatology
- Cardiovascular Medicine
- Pathology
Background:
- Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are common inflammatory conditions in older adults.
- Vasculitis is implicated in the pathogenesis of PMR.
- Both conditions require long-term management, often involving corticosteroids.
Observation:
- A patient with PMR and aortitis, initially responsive to steroid therapy, developed chest pain.
- The patient was diagnosed with a dissecting aortic aneurysm.
- Aortitis in the resected aorta showed mononuclear cell infiltration, including giant cells, around the vaso vasorum, disrupting the elastic lamina.
Findings:
- The case highlights a potential link between PMR/GCA and aortic dissection.
- Histological findings confirm vasculitic changes in the aorta.
- Aortic dissection can occur even with normal inflammatory markers (ESR, CRP) and after initial disease control.
Implications:
- PMR and GCA may signify an elevated risk for aortic dissection.
- Vigilant monitoring for aortic complications is essential in patients with PMR/GCA.
- Timely diagnosis and intervention for aortic dissection are critical, irrespective of active inflammatory symptoms.
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