Segmental Arterial Mediolysis: A Vasculitis Mimicker. A Single-Centre Experience

CLINICAL AND EXPERIMENTAL RHEUMATOLOGY(2020)

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摘要
Objective. Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown aetiology. It is non-atherosclerotic, non-inflatnnwtory, non-hereditary, non-infectious, large to medium-sized arteriopathy. SAM is a condition which in some circumstances behaves as a vasculitis mimicker and should be recognised in order to provide appropriate treatment and avoid unnecessary immune-suppressive therapy.Methods. We report a single-centre experience of 6 consecutive SAM cases (3 males and 3 females). A literature search of cases reported with SAM was performed and data summarised.Results. Abdominal or flank pain was the presenting symptom in 5 of the 6 patients. CT angiography (CIA) was the method of diagnosis in all 6 patients. 3 patients underwent therapeutic angiography; 2 with angiographic embolisaelan because of bleeding, and one patient needed a stew insertion because of left renal infarction. 2 patients underwent FDG-PET to rule out vasculitis. Serological tests were negative in all case, but C-reactive protein was elevated in 4 of them. 2 patients were treated with angiographic embolisation due to bleeding, 2 treated with anti-platelet therapy, one with scent insertion, and one with antihypertensive treatment. A medical literature review of 160 additional cases shows that abdominal or flank pain was the chief complaint in the vast majority of the cases. Renal and abdominal medium-sized arteries were the most commonly involved. CTA was the preferred method of diagnosis.Conclusion. SAM should be suspected in cases presenting with abdominal or flank pain. Angiographic features should be carefully studied by experienced radiologists to rule out vasculitis.
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segmental arterial mediolysis,computed tomographic angiography,vasculitis mimicker
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