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Published on: March 28, 2025
[Aortic dissection and annulo-aortic ectasia complicating systemic lupus erythematousus; report of a case]
1Department of Cardiovascular Surgery, Gunma Prefectural Cardiovascular Center, Maebashi, Japan.
Insights
Systemic lupus erythematosus patients on long-term steroids may develop aortic dissection and annulo-aortic ectasia due to myxomatous degeneration. Aortic root replacement is a viable treatment for severe aortic regurgitation in these cases.
Area of Science:
- Cardiovascular Medicine
- Rheumatology
- Surgical Pathology
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease.
- Long-term steroid therapy is a common treatment for SLE.
- Steroid-induced complications can affect various organ systems.
Observation:
- A 31-year-old male SLE patient on long-term steroids presented with asymptomatic aortic dissection and annulo-aortic ectasia.
- Severe aortic regurgitation was detected via echocardiogram.
- Magnetic resonance imaging confirmed the aortic abnormalities.
Findings:
- Aortic root replacement surgery was successfully performed.
- Histological examination of the aortic wall revealed myxomatous degeneration of the media.
- The patient recovered well, requiring temporary hemodiafiltration.
Implications:
- This case highlights a rare cardiovascular complication of long-term steroid use in SLE.
- Myxomatous degeneration may be an underlying mechanism for aortic pathology in this context.
- Early detection and surgical intervention are crucial for managing such severe aortic conditions.
Abstract:
A 31-year-old man with systemic lupus erythematosus had received long term steroid therapy. He had no compliant, but magnetic resonance imaging showed aortic dissection and annulo-aortic ectasia. Echocardiogram showed severe aortic regurgitation. Therefore aortic root replacement was performed. A histological study of the aortic wall demonstrated myxomatous degeneration in the media. He recovered uneventfully, except for receiving continious hemodia filtration during 4 days after the operation
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