A case of anti-neutrophil cytoplasmic antibody (MPO-ANCA) positive Wegener’s granulomatosis with pachymeningitis which is induced by a refractory otitis media

Abstract

    Hypertrophic pachymenigitis is a rare disease for which the pathogenesis is almost unknown. The following mechanisms have been blamed for causing hypertrophic pachymeningitis: infection, chronic inflammatory disease, trauma, medicine etc. In this case, hypertrophic pachymenigitis was complicated with MPO-ANCA positive Wegener’s granulomatosis. A 50-yearold woman presented with bilateral refractory otitis media and sinusitis. Histopathological examination of the operated specimen taken from otitis media revealed granulomatous inflammation with giant cell infiltration. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed a thickening of dura mater in the frontal cranial fossa and tentorium cerebelli. We diagnosed pachymeningitis associated with MPO-ANCA positive Wegener’s granulomatosis. Cyclophosphamide combined with prednisolone effectively improved the symptoms. In the case of MPO-ANCA positive hypertrophic pachymeningitis, we have to consider the differential diagnosis including of Wegener’s granulomatosis.