Eosinophilic Granulomatosis with Polyangiitis and Spinal Subdural Hematoma: A Case-Based Review of the Literature

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as a Churg-Strauss syndrome, is a rare systemic vasculitis. Central nervous system (CNS) involvement is described in rare cases. Acute spinal subdural hematoma (SSDH) is an uncommon condition presented with acute severe, radiating pain in the back area, and symptoms of spinal cord compression. The association of these two rare entities has been rarely reported so far. Case Presentation: A 32-year-old female known case of limited scleroderma for 12 years and EGPA for 6 years with presentation of eosinophilia, high P-ANCA, and skin vasculitis, was admitted to the rheumatology ward of Faghihi hospital related to Shiraz University of Medical Science in Iran (May 2016) due to new necrotizing vasculitis lesions on her hands, dyspnea, bilateral crackles, and eosinophilia. During this hospital admission, while receiving methylprednisolone, low dose aspirin, and prophylactic heparin, she developed severe upper back pain and neck rigidity and in her imaging studies, thoracic spinal subdural hematoma was seen. Conclusions: This article describes a case of EPGA presented with a non-traumatic acute subdural thoracic hematoma in addition to her classic symptoms along with limited scleroderma. This suggests that non-traumatic spontaneous acute spinal subdural hematomas should be considered in manifestations of EPGA and early recognition could be helpful for appropriate management.