Recurrent spinal subdural hematoma in granulomatosis with polyangiitis.

Abstract

Nervous system involvement is uncommon in granulomatosis with polyangiitis (GPA), a systemic autoimmune disease with episodes of necrotizing vasculitis. It is usually due to the compressive effect of dural or epidural masses. Spinal hemorrhagic presentation is exceptional. A 41-year-old woman diagnosed with GPA presented with three episodes of acute spinal subdural hematoma separated by eight years and ten months, respectively. The symptomatic debut was pain and paresis in all episodes. On all occasions, a lesion compatible with acute spinal subdural hematoma was diagnosed by magnetic resonance imaging (MRI). All episodes were treated conservatively with corticosteroids and immunosuppressants. The patient presented complete neurological recovery in the first two episodes. A mild residual left lower limb paresis remains after the last one. Follow-up MRI was performed after all episodes, and no focal intraspinal lesions were detected. Spinal subdural hemorrhage is a form of manifestation of GPA, either as a debut or in the course of the disease. We describe the third confirmed case of spontaneous spinal hemorrhage secondary to GPA published in the literature and the first with recurrence. Given the extraordinary response to immunosuppressive therapy, a high level of clinical suspicion is necessary to establish treatment as early as possible.