An autopsy case of Wegener's granulomatosis with pachymeningitis

A 61-year-old woman presented with high fever, headache and left facial palsy with diplopia. Histopathological examination of the biopsied specimens taken from nasal mucosa and kidney revealed a granulomatous angiitis with giant cell infiltration. Ga-DTPA-enhanced magnetic resonance imaging (MRI) revealed a thickening of dura mater in the middle cranial fossa and tentorium cerebelli. The observed left facial and occulomotor palsy was considered to be caused by pachymeningitis associated with Wegener's granulomatosis (WG). Cyclophosphamide combined with prednisolone effectively improved the symptoms. However, the patient died of acute interstitial pneumonitis, presumably caused by cyclophosphamide. The pathohistology obtained in the autopsy revealed a fibrous thickening of the dura mater in the left meningen with a segmental scarring of the arteries and a necrotizing arteritis in the kidney.