Polymyositis

Treatment of polymyositis with large doses of steroids is universally recommended and so clearly helpful, at least in the short term, that a controlled trial has never been considered necessary. A typical regimen87 is prednisolone 1 mg/kg daily for four to six weeks followed by gradual withdrawal at the rate of 5 mg of the daily dose per week. After the dose has reached 25-35 mg daily further reductions should be made more slowly, perhaps at 5 mg every two weeks. A wide range of doses are used and we prefer an alternate day dose, but others feel that this is not so effective.87 It is important to bring the disease under control before beginning the reduction and then to monitor the course of the disease closely with serial measurements of muscle strength and plasma creatine kinase concentrations. Although PE combined with cyclophosphamide has been reported to be beneficial in polymyositis,88,89 the usefulness of PE alone was not confirmed in a controlled trial in which three groups of 13 patients were treated with leucopheresis, PE, or sham PE.90 The authors of that trial claim an 80% power of detecting a minimal improvement in functional capacity. The trial did not answer the question whether PE followed by immunosuppression would provide a more rapid response than immunosuppression alone. Further exploration of this possibility would be worthwhile, as the PE group had a highly significant fall in plasma creatine kinase concentration compared with the sham PE group. Intravenous immunoglobulin was dramatically effective in two cases resistant to steroids and immunosuppressive drugs.91 Its use has been endorsed in dermatomyositis by the demonstration of benefit in a randomised trial.92 Immunosuppressive treatment with azathioprine, cyclophosphamide, or methotrexate has often been tried when steroids have failed. However standard oral immunosuppression does not have the rapid effect necessary to prevent or reverse the acute onset of respiratory failure. In desperation, total body low dose irradiation has sometimes been used and remissions have occurred in those cases that have been published.87

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