What are the treatment options for extramuscular manifestations of polymyositis?

Updated: Jan 05, 2021
  • Author: Mythili Seetharaman, MD; Chief Editor: Herbert S Diamond, MD  more...
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Treatment of extramuscular manifestations of polymyositis includes the following:

  • Constitutional symptoms, such as fever and fatigue - Usually respond to corticosteroids

  • Articular symptoms of polymyositis - Usually resolve with treatment of the myositis; some patients develop a rheumatoid-like arthropathy, which may require immunosuppressive treatment such as methotrexate

  • Interstitial lung disease - These patients may benefit from high-dose steroids and immunosuppressive treatment, especially cyclophosphamide. In a study of 30 patients and a historical comparison group of 21 patients, Shimojima et al reported improved outcomes when corticosteroids were combined with cyclosporine, administered orally or by continuous intravenous (IV) infusion for more severe cases; IV cyclophosphamide pulse therapy was added for exacerbations. [45]  In a study of 14 patients with dermatomyositis and acute interstitial pneumonia treated with infliximab, Chen et al reported satisfactory relief in the 10 patients given early treatment but treatment failure and death in those treated late. [46] Results of a study by Takada et al in 26 patients suggest that initial treatment with tacrolimus and corticosteroids may improve short-term mortality of patients with interstitial pneumonia. [47]

  • Cardiac abnormalities - May respond to corticosteroids; symptomatic arrhythmias require antiarrhythmic therapy, and symptomatic heart block is treated with placement of a pacemaker

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