Background:
Segmental arterial mediolysis (SAM) is a rare self-limiting non-atherosclerotic, non-inflammatory vasculopathy. SAM typically affects the visceral arteries of the abdomen to include the celiac, mesenteric, and renal arteries. SAM has a favorable prognosis in most cases with an asymptomatic course but can have mortality rates as high as 50% due to acute aneurysmal rupture. Very few cases of adverse long-term sequelae involving SAM have been described, and this report of chronic kidney disease represents a sentinel case illustrating that chronic disease can and does occur as a result of SAM and should be investigated for at follow-up.
Case presentation:
In this case report, we describe a case of a 45-year-old male with erectile dysfunction but without any readily identifiable risk factors for chronic kidney disease (CKD) or vasculopathy, who presented with bilateral renal infarction and parenchymal infarcts due to SAM and who subsequently developed CKD at follow-up. We conduct a mini-literature review that discusses the pathogenesis of SAM in the context of vasospastic diseases, as well as compares the outcomes of observation-only, versus medical-management, versus endovascular-interventions in patients with SAM.
Conclusion:
This is the first case to our knowledge of CKD occurring as an outcome of SAM without any preceding significant comorbidity, highlighting that whereas SAM is of itself rare and typically resolves, chronic disease can linger and should be evaluated for on follow-up. Further, we argue that radiological evidence of precursor vasospastic disease may exist in several locations apart from the index lesion and thus warrants wider whole-body radiographic exploration for lesions as an opportunity to prevent chronic sequelae as illustrated in this case report from occurring. Finally, a review of published case-series suggests that disease progression is less likely to occur after endovascular-intervention compared to observation-only or medical management and the risk of intervention vs conservative management should therefore be discussed with the patient.