The Therapeutic Effect of Azathioprine in Four Patients with Idiopathic Hypertrophic Pachymeningitis

ABSTRACT

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disease of unknown origin in which the recurrence is frequently observed despite an initial response to steroid therapy. Four patients, two men and two women aged 63 to 67 years, with severe headaches were evaluated by a brain MRI, and two patients were evaluated by follow up MRI receiving azathioprine therapy. All patients were given initial oral prednisolone 60mg or steroid pulse therapy followed by oral prednisolone and azathioprine therapy. Four patients improved with prednisolone but became steroid depen-dent. Azathioprine therapy permitted a reduction of the corticosteroid which may lead to clinical and radiological improvement. At present, high dose corticosteroid therapy is the treatment of choice, followed by immunosuppressive agents, such as azathioprine, if necessary. Further long-term follow-up studies of these patients are needed to clarify the outcome of this rare disease.