Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis that only exceptionally presents with pachymeningitis and multiple cranial neuropathies, which may hinder the early recognition of these atypical manifestations. Cases: We report two male patients, aged 61 and 67 years, who presented with headache, diplopia, and eyelid ptosis. Both showed pachymeningeal thickening on magnetic resonance imaging and involvement of multiple cranial nerves. They exhibited positive PR3-ANCA and marked cerebrospinal fluid hyperproteinorrachia without evidence of infection; additionally, in one case, biopsy ruled out neoplasia and IgG4-related disease. Both patients were treated with methylprednisolone and cyclophosphamide, achieving favorable outcomes with progressive neurological improvement. Conclusion: Although rare, GPA-associated pachymeningitis should be considered in patients with headache and cranial neuropathy. This case series provides novel Hispanic American evidence of a poorly characterized manifestation of the disease.

Keywords

  • Granulomatosis with Polyangiitis
  • Antibodies
  • Antineutrophil Cytoplasmic
  • Pachymeningitis
  • Cranial N

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