Differentiating between segmental arterial mediolysis and other arterial vasculopathies to establish an early diagnosis – a systematic literature review and proposal of new diagnostic criteria

ABSTRACT

Segmental arterial mediolysis (SAM) is a rare vascular disease, characterized by acute but transient vulnerability of the wall of medium-sized arteries. The most characteristic feature of SAM is its biphasic course: an injurious phase marked by acute weakness of the arterial wall leading to acute dissection and/or hemorrhage, followed by a reparative phase in which granulation tissue and fibrosis restore the injured arterial wall. Residual stenosis, aneurysms, and/or arterial wall irregularities may remain visible on future imaging studies. Differentiating between SAM and other arterial vasculopathies is difficult due to its similarities with many other vascular diseases, such as vasculitis, fibromuscular dysplasia, inherited connective tissue disorders, and isolated visceral artery dissection. In this systematic review, we provide an overview on SAM, with an emphasis on the differential diagnosis and diagnostic work-up. We propose new diagnostic criteria to help establish a prompt diagnosis of SAM, illustrated by case examples from our multidisciplinary vascular clinic.

KEYWORDS:

• Segmental arterial mediolysis
• vascular medicine
• vasculitis
• aneurysm
• dissection
• fibromuscular dysplasia

Declaration of financial/other relationships

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Acknowledgments

Part of this work was presented in the ‘Vascular Rounds’ conference in May 2023 in Roermond, The Netherlands. The authors thank the vascular medicine experts participating in this session for their critical input and helpful comments on the graded scoring system for segmental arterial mediolysis as discussed in this manuscript.

Author contributions

DJL van Twist, Y Appelboom: review conception, literature review, analysis and interpretation of results, figure preparation, and manuscript preparation. M Haagmans, R Riedl, O Yazar, LH Bouwman, G Mostard: literature review, analysis and interpretation of results, and manuscript review.

Supplementary material

Supplemental data for this article can be accessed online at https://doi.org/10.1080/00325481.2023.2288561.

Correction Statement

This article has been republished with minor changes. These changes do not impact the academic content of the article.

Additional information

Funding

This paper was not funded.