Hypertrophic pachymeningitis with anti-neutrophil cytoplasmic antibody (p-ANCA), and diabetes insipidus

We treated a patient with idiopathic cranial hypertrophic pachymeningitis a nd elevated serum titer or perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) reactive against myeloperoxidase. This 67-year-old man showed mul tiple cranial nerve-palsies, central diabetes insipidus (DI). and an intras ellar mass. DI and intrasellar mass had been present for 3 years, and DI ha d been well controlled by intranasal desmopressin. His nerve-palsies were m ost likely caused by thickened dura matter detected by the brain MRI. Granu loma may develop in the sella, and MRI findings in our patient are compatib le to it. Corticosteroid and oral cyclophosphamide therapy improved his neu rological symptoms and serum p-ANCA level with showing good correlation. DI improved temporally for 2 months. Few other cases of hypertrophic pachymen ingitis with elevated p-ANCA have been reported, however the etiology is un known. As p-ANCA antibodies have been detected in many of vasculitides, mic rovasculitis may be involved in some cases of idiopathic hypertrophic pachy meningitis.