Low-dose intravenous cyclophosphamide in systemic sclerosis: an open prospective efficacy study in patients with early diffuse disease

Scand J Rheumatol. Jan-Feb 2006;35(1):35-8. doi: 10.1080/03009740510026896.

Abstract

Objective: To investigate the efficacy of a treatment with low-dose intravenous cyclophosphamide (CYC) and low-dose prednisone in early diffuse cutaneous systemic sclerosis (dcSSc).

Methods: Patients with dcSSc and a disease duration <24 months consecutively admitted to a tertiary centre underwent a prospective 1-year study. They were treated with i.v. CYC 500 mg/pulses, 10 mg prednisone equivalent, and supportive therapy. Modified Rodnan skin score (mRss), Health Assessment Questionnaire-Disability Index (HAQ-DI), forced vital capacity (FVC), and diffusing lung capacity for CO (DLCO) were assessed as outcome measures. In addition, the nine Medsger severity scale scores were evaluated.

Results: mRss and DLCO significantly improved at both 6 (p = 0.002 and 0.012, respectively) and 12 months (p = 0.002 and 0.003, respectively). HAQ-DI showed a nearly significant reduction at 12 months (p = 0.06). Medsger's severity scores also improved for general condition (p = 0.001), peripheral vascular (p = 0.05), skin (p = 0.02), joint/tendon (p = 0.001), muscle (p = 0.05), and lung (p = 0.02). No treatment interruption was needed.

Conclusions: This preliminary study suggests a role for low-dose i.v. CYC in the treatment of early dcSSc. Controlled studies are warranted.

Publication types

  • Clinical Trial

MeSH terms

  • Adult
  • Antirheumatic Agents / administration & dosage
  • Antirheumatic Agents / therapeutic use
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use*
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Humans
  • Middle Aged
  • Patient Selection
  • Prednisone / therapeutic use
  • Scleroderma, Diffuse / drug therapy
  • Scleroderma, Diffuse / immunology
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / immunology
  • Severity of Illness Index
  • Time Factors
  • Treatment Outcome

Substances

  • Antirheumatic Agents
  • Cyclophosphamide
  • Prednisone