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Segmental arterial mediolysis and fibromuscular dysplasia: what comes first, the chicken or the egg?
E Tyler Hall1, Blake A Gibson2, Charles T Hennemeyer1
1Department of Medical Imaging, University of Arizona and Banner-University Medical Center, Tucson, AZ, USA.
Abstract:
Segmental arterial mediolysis (SAM) is a rare vasculopathy characterized by lysis of the outer media in splanchnic arteries and formation of dissecting pseudoaneurysms that may spontaneously rupture, leading to massive and often fatal intraabdominal hemorrhage. The pathogenesis of SAM is poorly understood. Healed SAM lesions closely resemble fibromuscular dysplasia (FMD), leading some authors to postulate that SAM represents a precursor to FMD despite distinct clinical differences between these two disorders. Herein, we present a 61-year-old woman with fatal SAM who showed histologic features in her aorta suggesting the opposite pathogenetic relationship, with an unclassified "FMD-like" arteriopathy preceding development of SAM.
Insights
Segmental arterial mediolysis (SAM) is a rare vasculopathy. This study suggests fibromuscular dysplasia (FMD)-like arteriopathy may precede SAM, challenging previous theories on their pathogenetic relationship.
Area of Science:
- Vascular Biology
- Pathology
- Medical Science
Background:
- Segmental arterial mediolysis (SAM) is a rare vasculopathy affecting splanchnic arteries, characterized by medial lysis and pseudoaneurysm formation.
- The pathogenesis of SAM remains poorly understood, with its relationship to fibromuscular dysplasia (FMD) debated.
- Healed SAM lesions share histological similarities with FMD, leading to hypotheses of SAM as a precursor to FMD.
Observation:
- A case of fatal SAM in a 61-year-old woman was investigated.
- Histological examination of the aorta revealed an unclassified "FMD-like" arteriopathy.
- This arteriopathy was observed to precede the development of SAM.
Findings:
- The findings suggest an "FMD-like" arteriopathy may precede SAM.
- This observation challenges the prevailing theory that SAM is a precursor to FMD.
- The study highlights a potential reversed pathogenetic relationship between these vascular disorders.
Implications:
- This case provides novel insights into the pathogenesis of SAM.
- Understanding the relationship between FMD-like arteriopathy and SAM is crucial for diagnosis and treatment.
- Further research is needed to elucidate the complex pathogenetic pathways involved in these rare vasculopathies.
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