Hypertrophic pachymeningitis with MPO-ANCA-positive vasculitis

A 75-year-old man presented with headache, right facial palsy, and left hemiparesis. Because of elevated myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) titers and findings from magnetic resonance imaging (MRI) which were compatible with hypertrophic pachymeningitis (HP), he was diagnosed with MPO-ANCA-positive HP. He was treated with the combination therapy of steroid and cyclophosphamide (CY), leading to good prognosis. We present a case of HP associated with MPO-ANCA-positive vasculitis and emphasize the importance of MPO-ANCA tests as a predictable factor for relapse of the disease in order to start earlier treatment for the disease.