Small-vessel vasculitis
Segmental arterial mediolysis: a vasculitis mimicker. A single centre experience
M. Abu Sneineh1, A. Farkas2, A. Natsheh3, G. Nesher4, G.S. Breuer5
- Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel.
- Department of Radiology, Shaare Zedek Medical Center, Jerusalem, Israel.
- Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem, Israel.
- Rheumatology Unit, Department of Medicine, Shaare Zedek Medical Center, Jerusalem; and Hebrew University School of Medicine, Jerusalem, Israel.
- 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.