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.

Keywords: Aneurysm growth; intracranial aneurysms; subarachnoid hemorrhage; systemic lupus erythematosus.

Figure 1

Computed tomography: (a) Axial noncontrast head CT demonstrating minimal interhemispheric SAH and multiple areas of acute infarct, most prominently in the anterior cerebral artery distribution. (b) Axial CTA with sagittal and 3D reconstructions demonstrating an 8 × 5 mm bilobed saccular aneurysm located at the junction of the left anterior cerebral anterior and anterior communicating artery (arrows)

Figure 2

Angiography and embolization: (a) Anterior-posterior projection of a contrast injection of the left internal carotid artery aneurysm that reveals a bilobed aneurysm (arrow) and proximal vasospasm (arrowhead). (b) Postembolization view showing complete occlusion of the aneurysm with a coil mass (arrow)

Figure 3

Prior MRI: Adjacent slices from axial T2-weighted MRI of the brain demonstrating normal anterior communicating artery complex, without evidence of intracranial aneurysm. Study performed 20 months prior to patient presentation (see Figures 1 and 2)