Efficacy and limitations of pulse cyclophosphamide therapy in polymyositis and dermatomyositis

J Clin Neuromuscul Dis. 2013 Jun;14(4):161-8. doi: 10.1097/CND.0b013e31828ee913.

Abstract

Objectives: To assess the therapeutic response of intravenous (IV) pulse cyclophosphamide therapy in polymyositis and dermatomyositis.

Methods: Data of 9 patients (M:F = 2:7) who received IV pulse cyclophosphamide therapy were retrospectively analyzed.

Results: The mean symptom duration was 11.33 ± 10.6 months (range, 2-34 months). The cohort comprised (1) primary idiopathic polymyositis (n = 1), (2) primary idiopathic dermatomyositis (n = 1), (3) childhood type associated with vasculitis (n = 1), and (4) associated with collagen vascular disease (n = 6). All patients improved and became clinically asymptomatic after a mean period of 12.33 ± 6.5 months (range, 4-24 months); 5 remained asymptomatic at the end of a median follow-up period of 22 months. All patients received concomitant steroid therapy, and in 6, steroids could be tapered after the initiation of IV pulse cyclophosphamide therapy.

Conclusions: In this cohort of polymyositis/dermatomyositis, treatment with IV pulse cyclophosphamide was associated with improvement; the therapeutic response was sustained in majority of the patients.

MeSH terms

  • Adolescent
  • Adult
  • Anti-Inflammatory Agents / therapeutic use
  • Cohort Studies
  • Cyclophosphamide / therapeutic use*
  • Dermatomyositis / complications
  • Dermatomyositis / drug therapy*
  • Desonide / therapeutic use
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Middle Aged
  • Polymyositis / complications
  • Polymyositis / drug therapy*
  • Young Adult

Substances

  • Anti-Inflammatory Agents
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Desonide