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Inicio Revista Colombiana de Reumatología (English Edition) Hypertrophic pachymeningitis in a patient with ANCA negative granulomatosis with...
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Vol. 27. Issue 4.
Pages 291-297 (October - December 2020)
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Vol. 27. Issue 4.
Pages 291-297 (October - December 2020)
Case Report
DOI: 10.1016/j.rcreue.2019.06.004
Hypertrophic pachymeningitis in a patient with ANCA negative granulomatosis with polyangiitis: A case report
Paquimeningitis hipertrófica en un paciente con granulomatosis con poliangeítis ANCA negativo: reporte de un caso
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Jorge Huaringa-Marceloa,b, Rubén Kevin Arnold Tapia-Orihuelac,
Corresponding author
ruben.tapia@unmsm.edu.pe

Corresponding author.
, David Loja-Oropezab,d
a Universidad Científica del Sur, Facultad de Ciencias de la Salud, Lima, Peru
b (Internal Medicine), Hospital Nacional Arzobispo Loayza, Lima, Peru
c Universidad Nacional Mayor de San Marcos, Facultad de Medicina de San Fernando, Lima, Peru
d Universidad San Martin de Porres, Lima, Peru
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Table 1. Laboratory results.
Table 2. CSF analysis.
Table 3. Laboratory results.
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Abstract

Granulomatosis with polyangiitis (GPA) is a vasculitic disease with an infrequent involvement of the central nervous system. This can lead, in rare cases, to hypertrophic pachymeningitis (HP), which is characterized by inflammation and fibrosis that cause a thickening of dura mater. At present, it is crucial to consider GPA in the differential diagnosis of elderly patients with intracranial hypertension. The case is presented of a 60-year-old male with progressive severe headache, vomiting, and wasting syndrome. Physical examination showed pallor, weight loss, and unilateral papilloedema. A gadolinium-enhanced brain MRI scan showed sinusitis, chronic otomastoiditis, and hypertrophic pachymeningitis. Finally, a meningeal biopsy concluded a necrotising granulomatous vasculitis compatible with GPA. However, PR3- and MPO-ANCA were negative. After corticosteroid therapy was initiated, the patient had a favorable outcome during his hospital stay.

Keywords:
Hypertrophic pachymeningitis
Vasculitis
Granulomatosis with polyangiitis
Intracranial hypertension
Resumen

La granulomatosis con poliangeítis (GPA) compromete excepcionalmente el sistema nervioso central, conllevando en raras ocasiones a una paquimeningitis hipertrófica (PH), caracterizada por inflamación y fibrosis, que originan un engrosamiento de la duramadre. Actualmente, su consideración es crucial en el diagnóstico diferencial de pacientes ancianos con hipertensión endocraneana. Presentamos el caso de un adulto de 60 años con cefalea severa progresiva, vómitos, papiledema unilateral y síndrome consuntivo en donde la resonancia magnética cerebral contrastada con gadolinio muestra sinusitis, otomastoiditis y PH. Finalmente, la biopsia de meninges reveló vasculitis granulomatosa necrosante de pequeños y medianos vasos compatible con GPA. Empero, PR3 y MPO-ANCA resultaron negativos. Se inició terapia con corticoides, presentando una evolución clínica favorable durante su hospitalización.

Palabras clave:
Paquimeningitis hipertrófica
Vasculitis
Granulomatosis con poliangeítis
Hipertensión endocraneana

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