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

Objective: Systemic lupus erythematosus (SLE) is an autoimmune disorder of multifactorial etiology with a broad range of clinical manifestations. Cerebral disorders such as psychosis, seizures and cerebrovascular accidents are commonly described in SLE patients. Subarachnoid hemorrhage (SAH) presents a higher incidence than in the general population. Saccular aneurysms are the most frequent cause, but uncommon forms of SAH had been repeatedly reported in SLE patients. Through three case examples we outline these uncommon patterns, which we hypothesized, might be responsible for the higher incidence of SAH in SLE patients. Methods: We present three patients previously diagnosed with SLE, who suffered a SAH and we perform a systematic literature review. Results: All three cases presented with a Fisher grade IV bleeding. One harbored a distal middle cerebral artery aneurysm, another had multiple aneurysms, and the youngest patient had no findings on the cerebral angiography. A focal vasculitic process seemed most likely responsible for the two aneurismal cases. The angiography negative case was attributed to severe SLE systemic complications and had an unexpected bad outcome. Literature review yielded 39 previously reported cases. Conclusions: The uncommon SAH patterns make etiological diagnosis challenging. Management can also be daunting due to both local and systemic consequences of SLE. Awareness of these uncommon patterns, management challenges, and higher likelihood of bad outcomes may help physicians dealing with this disease. Further research to unveil the mechanisms behind it is granted to fully understand SAH in SLE patients. (C) 2014 Elsevier B.V. All rights reserved.