Abstract
We experienced a case of otitis media with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and hypertrophic pachymeningitis (HP) followed by chronic subdural haematoma. He presented no pulmonary or renal manifestations or histology-proven vasculitis. Prednisolone treatment alone was inadequate, but the addition of intravenous cyclophosphamide (IVCY) was successful for treatment of not only the otitis media, but also the HP and subdural haematoma. HP is known to be frequently accompanied with ANCA-associated vasculitis (AAV), but subdural haematoma complicated with AAV, as in the present case, is rare. Our case suggests that aggressive treatment with IVCY is required for otitis media, HP and chronic subdural haematoma in patients with AAV although infectious adverse events should be carefully monitored.