Segmental arterial mediolysis

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Overview

Segmental arterial mediolysis (SAM) is a rare, non-inflammatory, non-atherosclerotic vascular disorder that affects medium-sized and large muscular arteries. It is characterized by lysis (breakdown) and degeneration of the outer media (the middle layer of the arterial wall), which can lead to arterial dissection, aneurysm formation, stenosis, and potentially life-threatening hemorrhage. SAM most commonly involves the abdominal visceral arteries, including the celiac, superior mesenteric, hepatic, splenic, and renal arteries, though other arterial beds may also be affected. Patients may present acutely with sudden abdominal or flank pain due to arterial dissection or rupture, intra-abdominal hemorrhage, or organ ischemia. Some cases are discovered incidentally on imaging performed for other reasons. The condition predominantly affects middle-aged to older adults, though cases have been reported across a range of ages. The exact cause of SAM remains unknown, though it is thought to involve a vasospastic mechanism or abnormal smooth muscle cell response rather than an inherited genetic defect. Treatment depends on the clinical presentation and severity. Acute hemorrhage or organ ischemia may require urgent endovascular intervention (such as embolization or stenting) or surgical repair. In stable patients with incidental findings, conservative management with close imaging surveillance may be appropriate. There is no specific medical therapy for the underlying arterial wall abnormality. Prognosis varies; some patients experience a self-limited course, while others may develop recurrent or progressive arterial lesions requiring ongoing monitoring.

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Segmental arterial mediolysis.

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No actively recruiting trials found for Segmental arterial mediolysis at this time.

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Specialists

1 foundView all specialists →
MU
Matthew W Urban
PLYMOUTH, MN
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Segmental arterial mediolysis.

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Community

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Common questions about Segmental arterial mediolysis

What is Segmental arterial mediolysis?

Segmental arterial mediolysis (SAM) is a rare, non-inflammatory, non-atherosclerotic vascular disorder that affects medium-sized and large muscular arteries. It is characterized by lysis (breakdown) and degeneration of the outer media (the middle layer of the arterial wall), which can lead to arterial dissection, aneurysm formation, stenosis, and potentially life-threatening hemorrhage. SAM most commonly involves the abdominal visceral arteries, including the celiac, superior mesenteric, hepatic, splenic, and renal arteries, though other arterial beds may also be affected. Patients may presen

How is Segmental arterial mediolysis inherited?

Segmental arterial mediolysis follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Segmental arterial mediolysis typically begin?

Typical onset of Segmental arterial mediolysis is adult. Age of onset can vary across affected individuals.

Which specialists treat Segmental arterial mediolysis?

1 specialists and care centers treating Segmental arterial mediolysis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.