{
  "abstract": "Introduction\n\nHypertrophic 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\n\nAn 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\n\nMPO 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\n\nMPO-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\n\nMPO-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.",
  "abstract_shown": true,
  "affiliations": [
    "Mental Health Department, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia.",
    "Research Group on Geriatrics and Gerontology, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia.",
    "Grupo de Resistencia Antibiótica de Manizales (GRAM), Manizales, Colombia.",
    "Internal Medicine Department, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia."
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 5,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Daniela Duque Montoya"
    },
    {
      "affs": [
        1,
        2
      ],
      "name": "Luis Felipe Aguirre García"
    },
    {
      "affs": [
        2
      ],
      "name": "Angélica María Vallejo Portilla"
    },
    {
      "affs": [
        3
      ],
      "name": "Camila García Ávila"
    },
    {
      "affs": [
        1
      ],
      "name": "José Fernando Gómez"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/9xs8y4hcqtmjh.bib",
  "cited_by": 0,
  "doi": "10.12890/2026_007403",
  "fulltext_url": "https://europepmc.org/articles/PMC13750775",
  "id": "9xs8y4hcqtmjh",
  "issns": [
    "2284-2594"
  ],
  "landing_url": "https://europepmc.org/article/MED/43011660",
  "language": "eng",
  "licence": "CC-BY-NC-ND",
  "licence_label": "CC BY-NC-ND",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by-nc-nd/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "openalex",
      "2745|Vasculitis and related conditions"
    ]
  ],
  "openalex": "W7223132931",
  "pdf_url": "https://europepmc.org/articles/PMC13750775?pdf=render",
  "pmcid": "PMC13750775",
  "pmid": "43011660",
  "published": "2026-10-21",
  "publisher": null,
  "ris": "https://research.draconitic.com/paper/9xs8y4hcqtmjh.ris",
  "sources": [
    {
      "first_seen": "2026-11-12T15:25:13Z",
      "native_id": "MED:43011660",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/43011660"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2745|Vasculitis and related conditions",
      "subfield": "general"
    }
  ],
  "title": "Sudden Bilateral Blindness Revealing MPO-ANCA-Associated Hypertrophic Pachymeningitis in an 82-Year-Old Woman.",
  "title_text": "Sudden Bilateral Blindness Revealing MPO-ANCA-Associated Hypertrophic Pachymeningitis in an 82-Year-Old Woman.",
  "type": "peer-reviewed",
  "updated_at": "2026-11-13T03:21:10Z",
  "url": "https://research.draconitic.com/paper/9xs8y4hcqtmjh",
  "venue": "European journal of case reports in internal medicine",
  "versions": []
}