Effective treatment of painful bone crises in type I gaucher's disease with high dose prednisolone.

Abstract

In type I Gaucher's disease, episodes of severe disabling bone pain, the so called bone crises, may be resistant to all analgesics, including narcotics. The demonstration of subperiosteal oedema on magnetic resonance imaging (MRI) led to an attempt to use steroids to relieve the oedema and thereby the pain. On eight occasions, five patients with documented bone crises received conventional dose steroids (20 mg/m2/day) with considerable shortening of the attacks. On six occasions five further patients received high dose methylprednisolone (30 mg/kg intravenously or 1 g/m2 orally daily for two days), which was followed by oral prednisone for three to five days on the last four occasions. In this later group, pain relief was evident within several hours. Three treatments were given on an ambulatory basis. The MRI scan of one of these patients showed no subperiosteal fluid collection five days after high dose steroids had been started, and on subsequent x ray examination, there was no periosteal elevation. This treatment should be considered in cases of Gaucher's disease with bone crises.