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Small-vessel vasculitis

 

Segmental arterial mediolysis: a vasculitis mimicker. A single centre experience


1, 2, 3, 4, 5

 

  1. Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel.
  2. Department of Radiology, Shaare Zedek Medical Center, Jerusalem, Israel.
  3. Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel.
  4. Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem; and Hebrew University School of Medicine, Jerusalem, Israel.
  5. Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem; and Hebrew University School of Medicine, Jerusalem, Israel. gbreuer@szmc.org.il

CER12554
2020 Vol.38, N°2 ,Suppl.124
PI 0148, PF 0154
Small-vessel vasculitis

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PMID: 31820718 [PubMed]

Received: 02/07/2019
Accepted : 14/10/2019
In Press: 09/12/2019
Published: 21/05/2020

Abstract

OBJECTIVES:
Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown aetiology. It is non-atherosclerotic, non-inflammatory, 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 (CTA) was the method of diagnosis in all 6 patients. 3 patients underwent therapeutic angiography; 2 with angiographic embolisation because of bleeding, and one patient needed a stent 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 stent 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.
CONCLUSIONS:
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.

Rheumatology Article