Cerebral transmural angiitis and ruptured cerebral aneurysms in patients with systemic lupus erythematosus

Abstract

Two cases of cerebral transmural angiitis and ruptured aneurysm in patients with established systemic lupus erythematosus are presented. A 22-year-old woman with a 4-year history of systemic lupus erythematosus was found to have a ruptured cerebral aneurysm at the trifurcation of the middle cerebral artery. She died 10 days after admission because of the brain swelling. Necropsy showed remarkable brain edema and focal transmural angiitis at the site of the ruptured aneurysm. Another 29-year-old woman with a 3-year history of systemic lupus erythematosus had an acute subdural hematoma resulting from a ruptured aneurysm of the right anterior cerebral artery. She was surgically treated. The ruptured aneurysm was resected and examined histologically. The transmural angiitis was demonstrated. This report describes two radiographically and pathologically confirmed cases of central nervous system lupus producing focal angiitis of a cerebral artery with secondary aneurysm formation.

Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: two case reports and a review of the literature

“…The development of SAH is affected not only by SLE itself but also by its complications and therapies, in addition to other established causes of SAH. Several case reports have histologically demonstrated inflammation in intracranial arterial and arteriolar walls of SLE patients with SAH [18,19,26,29,37]. This suggests that some SLE patients with SAH (these patients are thought to have high disease activity) have intracranial vasculitis.…”

“…To the best of our knowledge, 55 Japanese SLE patients with SAH, excluding cases involving secondary leakage from an intracerebral hemorrhage, have been reported to date [5,[12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29]. We analyzed 57 Japanese SLE cases with SAH, including our two cases.…”

Successful Flow Reduction Treatment for a Middle Cerebral Artery Aneurysm in a Patient With Systemic Lupus Erythematosus

“…The etiology of cerebral aneurysm in SLE is still controversial, although vasculitis has been pathologically proven in solitary berry aneurysms. 9,18) In our case, the walls of the aneurysm and dilatation of the inferior trunk M 2 were very thin in some areas. Even fusiform aneurysm is liable to rupture in SLE patients, and outcome tends to be poor, 2,16) so we selected an aggressive treatment such as proximal clipping, followed by STA-MCA bypass, in addition to neck clipping.…”

Subarachnoid hemorrhage in systemic lupus erythematosus: Systematic review and report of three cases

“…Inflammation of arteries narrows the arterial lumen, compromises cerebral blood flow, and causes a variety of atherosclerotic-like symptoms leading to ischemia and hemodynamic stress, which have also been previously identified as aneurysms inducers. Furthermore, focal vascultitis could be the cause of the uncommon aneurysms in unusual locations that are frequently described in patients with SLE [10,[22][23][24]. However, only three authors have been able to prove anatomopathological findings supporting focal transmural angiitis in fusiform aneurysms [14,19,24].…”