Abstract

HUARINGA-MARCELO, Jorge; TAPIA-ORIHUELA, Rubén Kevin Arnold  and  LOJA-OROPEZA, David. Hypertrophic pachymeningitis in a patient with ANCA negative granulomatosis with polyangiitis: A case report. Rev.Colomb.Reumatol. [online]. 2020, vol.27, n.4, pp.291-297.  Epub Aug 15, 2021. ISSN 0121-8123.  https://doi.org/10.1016/j.rcreu.2019.06.003.

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.

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