DOI: 10.12890/2026_007403
Sudden bilateral blindness revealing MPO-ANCA-associated hypertrophic pachymeningitis in an 82-year-old woman
Authors
aguirregarcialuis@gmail.com
Keywords
Vasculitis, central nervous system, ANCA-associated vasculitis, myeloperoxidase, bilateral blindness, glucocorticoidsAbstract
Introduction: Hypertrophic pachymeningitis is a rare inflammatory thickening of the dura mater, complicating an estimated 1–3% of ANCA-associated vasculitis (AAV), usually PR3-ANCA disease with systemic involvement. Isolated meningeal disease with normal inflammatory markers is exceptional and easily mistaken for a non-inflammatory or ischaemic optic neuropathy.
Case description: An independent 82-year-old woman presented with 48 hours of bilateral visual loss after nine days of severe, non-postural occipital headache. She had no light perception, non-reactive pupils and optic disc pallor, without renal, pulmonary, cutaneous or sinonasal manifestations. Chest computed tomography, erythrocyte sedimentation rate and creatinine were normal. Contrast-enhanced magnetic resonance imaging showed diffuse pachymeningeal thickening with intense dural enhancement. Cerebrospinal fluid was acellular, with elevated protein and negative microbiology, including two Mycobacterium tuberculosis PCR assays. Anti-MPO exceeded 400 RU/ml, with negative anti-PR3, low C3 and C4, and normal IgG4, consistent with microscopic polyangiitis. Intravenous methylprednisolone 500 mg/day, started four days after blindness onset, was followed by recovery to counting fingers at 30 cm in the right eye by day 9.
Discussion: MPO specificity, isolated blindness and an absent acute-phase response made this presentation atypical. Visual loss probably reflected dural compression and hypoperfusion of the optic nerves rather than infarction, possibly explaining partial recovery. An ejection fraction of 30% favoured rituximab over cyclophosphamide.
Conclusion: MPO-ANCA-associated vasculitis may be confined to the dura, with normal acute-phase reactants. ANCA testing should accompany rather than follow the work-up for dural enhancement, and glucocorticoids should not await the result: functional reserve, not age, determines treatment intensity.
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