Sudden Bilateral Blindness Revealing MPO-ANCA-Associated Hypertrophic Pachymeningitis in an 82-Year-Old Woman.
4 affiliations
- 1Mental Health Department, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia.
- 2Research Group on Geriatrics and Gerontology, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia.
- 3Grupo de Resistencia Antibiótica de Manizales (GRAM), Manizales, Colombia.
- 4Internal Medicine Department, Faculty of Health Sciences, Universidad de Caldas, Manizales, Colombia.
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
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.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.
Related
- Anti-GQ1b-Positive Bickerstaff Brainstem Encephalitis Complicated by Diffuse Hypertrophic Pachymeningitis: A Case Report
- Insufficient Dural Thickness Recovery in Acute Treatment Predicts Subsequent Relapses in Idiopathic Hypertrophic Pachymeningitis
- Bilateral Vision Loss due to Fulminant Hypertensive Optic Neuropathy Mimicking Cortical Blindness in a Patient with Co-existing Vestibular Schwannoma: A Case Report
- Cerebral Venous Sinus Thrombosis With Bilateral Blindness in Systemic Lupus Erythematosus: A Case Report.
- Polymyalgia Rheumatica or Something Else? Bilateral Shoulder, Arm, and Thigh Pain with Diffuse Marrow Edema Revealing B-cell Acute Lymphoblastic Leukemia
BibTeX
@article{montoya2026sudden,
title = {{Sudden Bilateral Blindness Revealing MPO-ANCA-Associated Hypertrophic Pachymeningitis in an 82-Year-Old Woman.}},
author = {Daniela Duque Montoya and Luis Felipe Aguirre García and Angélica María Vallejo Portilla and Camila García Ávila and José Fernando Gómez},
journal = {European journal of case reports in internal medicine},
year = {2026},
month = oct,
doi = {10.12890/2026_007403},
url = {https://europepmc.org/article/MED/43011660},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Sudden Bilateral Blindness Revealing MPO-ANCA-Associated Hypertrophic Pachymeningitis in an 82-Year-Old Woman.
AU - Daniela Duque Montoya
AU - Luis Felipe Aguirre García
AU - Angélica María Vallejo Portilla
AU - Camila García Ávila
AU - José Fernando Gómez
PY - 2026
DA - 2026/10/21
JO - European journal of case reports in internal medicine
DO - 10.12890/2026_007403
UR - https://europepmc.org/article/MED/43011660
AB - 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.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.
SN - 2284-2594
ER -