E-ISSN: 1019-5157
ISSN: 2651-5024
Case Report
MPO - ANCA - Related Thoracic Spinal Hypertrophic Pachymeningitis: A Case Report
Keiichiro Torigoe✉ ,
Takehiro Uno ,
Sho Takata ,
Toshihiro Ogiwara ,
Sohsuke Yamada ,
Yasuhiko Hayashi
DOI: 10.5137/1019-5149.JTN.50048-25.3
Article in Press
Corresponding Author:
Keiichiro Torigoe (torigoe@kanazawa-med.ac.jp)
Abstract
Background:
Hypertrophic spinal pachymeningitis (HSP) is a rare inflammatory disorder characterized by progressive thickening of the spinal dura mater. The diagnosis requires careful exclusion of infectious, autoimmune, and neoplastic etiologies. Although HSP associated with myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA) has been reported, cases without systemic vasculitis remain exceedingly rare.
Case Description:
A 69-year-old man presented with back pain, weight loss, and progressive paraparesis. Magnetic resonance imaging revealed diffuse thickening of the thoracic dura mater causing spinal cord compression. Laboratory evaluation demonstrated elevated MPO-ANCA levels. Emergency decompressive laminectomy was performed, and hypertrophied granulomatous tissue and thickened dura were resected for biopsy. Histopathological examination showed fibrotic tissue with nonspecific chronic inflammation without evidence of vasculitis. Postoperatively, corticosteroid therapy resulted in marked neurological improvement, and follow-up imaging at one year demonstrated complete resolution of dural thickening without recurrence.
Conclusion:
MPO-ANCA-related HSP should be considered in patients presenting with rapidly progressive thoracic myelopathy and radiological evidence of dural hypertrophy. Early decompression with biopsy is essential for definitive diagnosis and exclusion of infection. Prompt initiation of corticosteroid therapy can prevent irreversible neurological sequelae and achieve favorable long-term outcomes.
Hypertrophic spinal pachymeningitis (HSP) is a rare inflammatory disorder characterized by progressive thickening of the spinal dura mater. The diagnosis requires careful exclusion of infectious, autoimmune, and neoplastic etiologies. Although HSP associated with myeloperoxidase–antineutrophil cytoplasmic antibody (MPO-ANCA) has been reported, cases without systemic vasculitis remain exceedingly rare.
Case Description:
A 69-year-old man presented with back pain, weight loss, and progressive paraparesis. Magnetic resonance imaging revealed diffuse thickening of the thoracic dura mater causing spinal cord compression. Laboratory evaluation demonstrated elevated MPO-ANCA levels. Emergency decompressive laminectomy was performed, and hypertrophied granulomatous tissue and thickened dura were resected for biopsy. Histopathological examination showed fibrotic tissue with nonspecific chronic inflammation without evidence of vasculitis. Postoperatively, corticosteroid therapy resulted in marked neurological improvement, and follow-up imaging at one year demonstrated complete resolution of dural thickening without recurrence.
Conclusion:
MPO-ANCA-related HSP should be considered in patients presenting with rapidly progressive thoracic myelopathy and radiological evidence of dural hypertrophy. Early decompression with biopsy is essential for definitive diagnosis and exclusion of infection. Prompt initiation of corticosteroid therapy can prevent irreversible neurological sequelae and achieve favorable long-term outcomes.
Keywords
MPO - ANCA
spine
pachymeningitis
corticosteroids