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[An autopsy case of segmental mediolytic arteritis (SMA) accompanied with microscopic polyarteritis nodosa]
M R Ito1, H Ohtani, Y Nakamura
1Department of Pathology, School of Medicine, Tohoku University, Sendai-city.
Abstract:
Segmental Mediolytic Arteritis (SMA) is a very rare arterial lesion which is limited in adults to the involvement of the intra-abdominal muscular arteries. The pathology is characterized by segmental disruption of the arterial media which leads segmental mediolysis, with subsequent dissecting aneurysm or rupture. A 73-year-old man was admitted to a hospital because of high fever, general fatigue and weight loss. These symptoms were resistant to antibiotic therapy, and soon after, renal insufficiency developed. Three months after the onset of symptoms, he died suddenly of hemorrhagic shock. Autopsy revealed rupture of the splenic artery and systemic necrotizing arteritis in the small-sized arteries of liver, pancreas and kidneys, as well as in the connective tissues around the adrenal glands. Histopathology of the splenic artery was consistent with SMA, and that of the systemic vascular lesions, with microscopic polyarteritis nodosa. Focal glomerular lesions characteristic of crescentic and/or granulomatous glomerulonephritis were present. A developmental mechanism for SMA is discussed with respect to this case together with a review of previous reports of this disease.
Insights
Segmental Mediolytic Arteritis (SMA) is a rare arterial disease causing vessel wall disruption. This case highlights SMA
Area of Science:
- Vascular Pathology
- Nephrology
- Rheumatology
Background:
- Segmental Mediolytic Arteritis (SMA) is a rare arterial disease affecting intra-abdominal muscular arteries.
- It is characterized by segmental disruption of the arterial media, potentially leading to dissecting aneurysms or rupture.
Observation:
- A 73-year-old man presented with fever, fatigue, and weight loss, unresponsive to antibiotics.
- Renal insufficiency developed, and the patient died from hemorrhagic shock three months later.
- Autopsy revealed splenic artery rupture, systemic necrotizing arteritis in multiple organs, and glomerular lesions.
Findings:
- Histopathology confirmed Segmental Mediolytic Arteritis in the splenic artery.
- Systemic vascular lesions were consistent with microscopic polyarteritis nodosa.
- Focal glomerular lesions indicated crescentic and/or granulomatous glomerulonephritis.
Implications:
- This case provides insights into the developmental mechanisms of Segmental Mediolytic Arteritis.
- It underscores the association between SMA, systemic necrotizing arteritis, and renal pathology.
- Further research is needed to understand and manage this rare condition.