Rapid aneurysm growth and rupture in systemic lupus erythematosus

Abstract

Background:Subarachnoid hemorrhage (SAH) due to intracranial aneurysm rupture is a major neurosurgical emergency associated with significant morbidity and mortality. Rapid aneurysm growth is associated with rupture. Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder whose complications can include cerebral vasculitis and vasculopathy. Intracranial aneurysms are not known to occur more frequently in SLE patients than the general population; however, aneurysm growth rates have not been studied in SLE.Case Description:We present a 43-year-old female with SLE on prednisone, hydroxychloroquine, and azathioprine with moderate disease activity who presented with severe, acute-onset headache and was found to have Hunt and Hess grade II SAH due to rupture of an 8 mm saccular anterior communicating artery (ACoA) aneurysm. The patient developed severe vasospasm, re-ruptured, and was taken for angiography and embolization, which was challenging due to a high degree of vasospasm and arterial stenosis. Review of imaging from less than 2 years prior demonstrated a normal ACoA complex without evidence of an aneurysm.Conclusion:We review the literature and discuss the risk factors and pathophysiology of rapid aneurysm growth and rupture, as well as the pathologic vascular changes associated with SLE. Although SLE patients do not develop intracranial aneurysm at an increased rate, these changes may predispose them to higher incidence of growth and rupture. This possibility-coupled with increased morbidity and mortality of SAH in SLE-suggests that SAH should be considered in SLE patients presenting with headache, and advocates for more aggressive treatment of SLE patients with unruptured aneurysms.

Does Systemic Lupus Erythematosus Increase the Risk of Procedure-Related Complication in Endovascular Treatment of Intracranial Aneurysm?

“…Cerebral aneurysms associated with SLE are more likely to grow rapidly and consequently rupture, compared to cerebral aneurysms in the general population. 4 Therefore, when an aneurysm is present and requires occlusion in patients with SLE, it should be considered for more aggressive treatment, especially if a growth or related symptom of aneurysm is observed, as in our case.…”

Fulminant Vasculitis Associated with Extracranial Dissections and Occlusion, Ischemic Strokes, and Aneurysm Rupture: Case Report and Review of the Literature

“…3,7 These patients have a worse prognosis, higher mortality, and a greater Hunt and Hess grade. 3,5,[7][8][9][10][11][12][16][17][18][19] One reported patient with SLE and aneurysmrelated subarachnoid hemorrhage presented with rapidly progressive increase in size of the intracranial aneurysm with subsequent rerupture. 3 Rarely, tuberculosis and CNS aspergillosis have been implicated in vasculitis leading to aneurysm formation with subsequent rupture (Table 1).…”

“…[2][3][4] Angiography plays a critical role in the diagnosis of CNS vasculitis, 1,5 but as a radiographic intervention, it does not provide information to suggest whether the changes seen represent primary or secondary vasculitis or even a nonvasculitic case of segmental arteriopathy. Confirmation generally requires a brain biopsy, which was not performed in our case.…”

Multiple cerebral aneurysms: a rare presentation in systemic lupus erythematosus

“…SLE is a chronic autoimmune disease with multiple pathologies that can affect every organ system of body including central nervous system (CNS). 1 CNS systemic lupus erythematosus (SLE) occurs in 24-51% of patients with psychosis and seizures which are among the diagnostic criteria of SLE. 2,3 Intracerebral aneurysms and subarachnoid haemorrhage (SAH) are one of the comparatively rare manifestations of CNS-SLE occurring in upto 3.9% of cases.…”