Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis

Hypertrophic cranial pachymeningitis (HCP) is a rare inflammatory disease of unknown origin in which recurrence is frequently observed in spite of the initial response to steroid therapy. Three patients, 1 man and 2 women, aged 63, 66, and 67 years, with severe intractable headache were evaluated by brain MRI. All patients were initially given prednisolone (60 mg/day, oral) or dexamethasone (20 mg/day, i.v.), and followed by long-term (at least 1 year) azathioprine therapy. All patients were evaluated by follow-up laboratory tests and brain MRI study, and completed the 2-year follow-up period. Symptoms including headache were initially improved with corticosteroid therapy, but patients became steroid-dependent. Azathioprine administration in these steroid-dependent patients permitted the complete cessation of corticosteroid and led to the clinical and radiological recovery. In conclusion, initial high-dose corticosteroid administration followed by long-term azathioprine therapy may be the ideal treatment of HCP at present.