Work

Sudden bilateral blindness revealing MPO-ANCA-associated hypertrophic pachymeningitis in an 82-year-old woman

Year: 2026

Type: article

Abstract: 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: 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. LEARNING POINTS: MPO-ANCA-associated vasculitis may present with complete bilateral blindness as the sole initial manifestation, even in the absence of renal, pulmonary, cutaneous or sinonasal involvement. Normal acute-phase reactants, including a normal erythrocyte sedimentation rate, and an otherwise unrevealing systemic evaluation do not exclude the diagnosis.Diffuse dural enhancement is non-specific; therefore, ANCA testing with anti-MPO and anti-PR3 assays should be performed alongside the evaluation for infectious, IgG4-related, neoplastic and intracranial hypotension-related causes. Hypertrophic pachymeningitis remains a diagnosis of exclusion.Suspected inflammatory pachymeningitis with visual loss should be treated as an ophthalmological emergency, as in giant cell arteritis; frailty and functional reserve, not chronological age, should determine treatment intensity, even when comorbidity constrains the choice of immunosuppressant.

Source: European Journal of Case Reports in Internal Medicine

DOI: 10.12890/2026_007403

Authors: Daniela Duque Montoya, Luis Felipe Aguirre García, Angélica María Vallejo Portilla, Camila García Ávila, José Fernando Gómez

Institution: Universidad de Caldas

Language: English

Topic: Vasculitis and related conditions

Subfield: Rheumatology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $323