A case of microscopic polyangiitis presenting with acute spinal subdural hemorrhage

Abstract

This report describes a case of a 62-year-old woman with microscopic polyangiitis (MPA) who developed acute spinal subdural hemorrhage. MPA was confirmed by positive autoantibodies to myeloperoxidase and focal segmental necrotizing and pauci-immune crescentic glomerulonephritis on renal biopsy. She did not recover from paraplegia due to acute spinal subdural hemorrhage, despite decompression operation and aggressive immunosuppression. Although spontaneous spinal hemorrhage in MPA patients is very rare, the prognosis for such patients is poor. Considering the possibility of ongoing vasculitis activity in extra-renal organs, clinicians should be very cautious to attenuate the strength of immunosuppressant drugs, even in patients with chronic or irreversible renal pathology.

Keywords: Microscopic polyangiitis; Spinal; Spinal subdural hematoma.

Figure 1. Magnetic resonance images

(A) The sagittal T1-weighted image reveals prominent spinal cord compression at the level of the mid-thoracic spine (from T5 to T9). (B, C) The axial T2- and T1-weighted image shows subdural fluid compressing the cauda equina anteriorly at the level of L4 to L5.

Figure 2. Kidney biopsy finding

Focal segmental necrotizing crescentic glomerulonephritis with moderate tubular atrophy and interstitial fibrosis are shown (H&E stain, ×100).

Figure 3. Clinical course and treatment of the patient

CMV, cytomegalovirus; CPHO, cyclophosphamide (mg/day); CRRT, continuous renal replacement therapy; HD, hemodialysis; MPD, methylprednisolone (mg/day); PD, prednisolone (mg/day).