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.

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.

Related Concept Videos

Peripheral Artery Disease I: Introduction01:30

Peripheral Artery Disease I: Introduction

Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
601
Atherosclerosis I: Introduction01:30

Atherosclerosis I: Introduction

Atherosclerosis is a progressive disorder characterized by the buildup of plaques on the arterial inner wall, causing them to narrow and harden over time. These plaques comprise lipids, calcium, blood components, carbohydrates, and fibrous tissue. The process primarily affects the intima of large and medium-sized arteries, reducing blood flow in any artery.Etiology and risk factorsThe cause of atherosclerosis is multifactorial, involving a complex interplay among endothelial injury, lipid...
1.9K
Coronary Artery Disease II: Pathophysiology01:26

Coronary Artery Disease II: Pathophysiology

Coronary Artery Disease (CAD) originates from a series of events that impair the function of coronary arteries, the blood vessels responsible for delivering oxygen-rich blood to the heart muscle. The pathophysiology of CAD is closely linked to atherosclerosis, a chronic inflammatory and lipid-driven condition affecting the vascular endothelium.1. Endothelial DamageThe process begins with damage to the vascular endothelium, which serves as a protective barrier between the blood and the vessel...
955
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
1.3K
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
912
Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
2.6K