Two Cases of Catastrophic AAA Rupture in Young Women with Systemic Lupus Erythematosus

Abstract

We present two cases of young women with a past medical history significant for systemic lupus erythematosus (SLE), who presented to the Emergency Department with a ruptured abdominal aortic aneurysm (AAA). These cases are of particular interest because the patients did not fit the typical demographic for patients who present with a ruptured AAA. Based on these cases and a review of the relevant literature, ED providers should maintain a higher index of suspicion for AAA rupture in patients with autoimmune diseases, especially SLE.

A Sinister Gut Feeling

“…highlighting the need to maintain a higher index of suspicion for aortic aneurysms in any individual with SLE, irrespective of age, who presents with symptoms such as severe abdominal, flank, back pain, syncope, or gastrointestinal bleeding. [ 15 ]…”

"Pathophysiology of Vascular Manifestations in Systemic Lupus Erythematosus"

“…Apart from the higher incidence and mortality of aneurysm in SLE patient's vs the general population, the age of onset is significantly lower among the former: In two meta-analyses, the median age for onset of AA was around 45 years [72,73], which is around 20 years younger compared to the general population [74]. In addition to that, patients with SLE complicated by abdominal AA are struck with disease around 30 years earlier [56].…”

“…Sites of aneurysm often display atherosclerosis, which increases the likelihood to rupture plaques as a result of aneurysmatic dilatation [246]. As atherosclerosis and abdominal AA are also positively correlated among both the general population and SLE patients [56,69,72,247], this pathomechanism might be similar to such of non-SLE-related abdominal AA. This is supported by the fact that abdominal AAs are more likely to occur in hypertensive, older SLE patients [71], which might lead to the assumption that general risk factors of abdominal AA also apply to SLE patients.…”

“…In adult-onset SLE patients, numerous cases of aneurysm or dissection of variable severity and of different vessels have been reported with onset at strikingly young ages, among which thoracic [52][53][54]54] and abdominal aortic [54][55][56], coronary [57,58], pulmonary [59], carotid [60], basilar [61], cerebral [62,63], hepatic [64] and mesenteric [65] artery aneurysms. Some cases have been reported as early as in childhood [66] and adolescence [67,68].…”

“…This is supported by the fact that abdominal AAs are more likely to occur in hypertensive, older SLE patients [71], which might lead to the assumption that general risk factors of abdominal AA also apply to SLE patients. Therefore, abdominal AA might be fueled by secondary hypertension due to damage of renal vasculature in SLE, which has also been demonstrated as an independent risk factor for the development of aneurysm [56].…”