Segmental arterial mediolysis

Anil Kumar Pillai, Shams I. Iqbal, Raymond W. Liu, Niranjan Rachamreddy, Sanjeeva P. Kalva

Research output: Contribution to journalArticle

33 Scopus citations

Abstract

Segmental arterial mediolysis (SAM) is an uncommon, nonatherosclerotic, noninflammatory, large- to medium-sized arteriopathy first described in 1976. It is characterized histologically by vacuolization and lysis of the outer arterial media leading to dissecting aneurysms and vessel rupture presenting clinically with self-limiting abdominal pain or catastrophic hemorrhages in the abdomen. Patients of all ages are affected with a greater incidence at the fifth and sixth decades. There is a slight male predominance. Imaging findings overlap with inflammatory vasculitis, collagen vascular disease, and fibromuscular dysplasia. The presence of segmental dissections involving the celiac, mesenteric, and/or renal arteries is the key distinguishing features of SAM. Inflammatory markers, genetic tests for collagen vascular disorders, and hypercoagulable studies are negative. Anti-inflammatory agents and immunosuppressants are not effective. A mortality rate of 50 % has been attributed to the acute presentation with aneurysmal rupture necessitating urgent surgical or endovascular treatments; in the absence of the acute presentation, SAM is a self-limiting disease and is treated conservatively. There are no established guidelines on medical therapy, although optimal control of blood pressure is considered the main cornerstone of medical therapy. The long-term prognosis is not known.

Original languageEnglish (US)
Pages (from-to)604-612
Number of pages9
JournalCardiovascular and Interventional Radiology
Volume37
Issue number3
DOIs
StatePublished - Jun 2014

Keywords

  • Fibromuscular displasia
  • Segmental arterial mediolysis
  • Vasculopathy

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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    Pillai, A. K., Iqbal, S. I., Liu, R. W., Rachamreddy, N., & Kalva, S. P. (2014). Segmental arterial mediolysis. Cardiovascular and Interventional Radiology, 37(3), 604-612. https://doi.org/10.1007/s00270-014-0859-4