Hypertrophic pachymeningitis, associated with eosinophilic granulomatosis with polyangiitis, and ANCA-negative serology
  • Daniel Arturo Martínez-Piña
    Neurology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico
  • Ana Laura Calderón-Garcidueñas
    Neuropathology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico
  • Elizabeth Gama-Lizárraga
    Neurological Emergency Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico
  • Kevin Giuseppe Enríquez-Peregrino
    Neurology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico
  • José María Curiel-Zamudio
    Neuroradiology Department, Instituto Nacional de Neurología y Neurocirugía Manuel Velasco Suárez, Mexico City, Mexico

Keywords

Headache, bilateral sensorineural hearing loss, hypertrophic pachymeningitis, eosinophilic granulomatosis with polyangiitis

Abstract

Background: Hypertrophic pachymeningitis (HP) is a disease with diverse aetiologies, including the autoimmune one, either associated with antineutrophil cytoplasmic antibodies or immunoglobulin G4.
Case description: A 65-year-old woman with a history of systemic arterial hypertension, presented with intense progressive headaches. HP and hemispheric vasogenic oedema were observed by nuclear magnetic resonance (NMR) study. During the six months before the headache, she had developed progressive hearing loss which she attributed to age. A biopsy of dura mater showed necrotising vasculitis with peripheral inflammatory infiltrate, made up of accumulations of epithelioid cells and multinucleated giant cells, and abundant eosinophils. A final diagnosis of HP with eosinophilic granulomatosis with polyangiitis (EGPA) was made.
Discussion: The patient had eosinophilic granulomatosis with polyangiitis (EGPA) histology, ANCA-negative serology and HP. This case is important because it shows that EGPA seems to have a spectrum of clinical diseases, including HP with negative serology, and bilateral sensorineural hearing loss.
Conclusion: We are facing a wide spectrum of EGPA, breaking the paradigm of only systemic involvement.

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References

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    Published: 2024-06-28
    Issue: 2024: LATEST ONLINE (view)

    How to cite:
    1.
    Martínez-Piña DA, Calderón-Garcidueñas AL, Gama-Lizárraga E, Enríquez-Peregrino KG, Curiel-Zamudio JM. Hypertrophic pachymeningitis, associated with eosinophilic granulomatosis with polyangiitis, and ANCA-negative serology. EJCRIM 2024;11 doi:10.12890/2024_004595.