Efficacy of Cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids

Sci Rep. 2017 Jul 21;7(1):6195. doi: 10.1038/s41598-017-06520-5.

Abstract

Aim to evaluate the efficacy and safety of glucocorticoid monotherapy vs combination therapy of cyclophosphamide (CYC) for IgG4 related disease (IgG4-RD). 102 newly diagnosed IgG4-RD patients were enrolled and assigned to 2 groups: Group I was prednisone monotherapy (0.5-1.0 mg/kg.d, tapered gradually) and Group II was glucocorticoid and CYC (50-100 mg per day). Patients were assessed at different periods. Primary end point was relapse rate; secondary end points included response, remission rate and adverse effects. 52 patients were in Group I and 50 in Group II. At 1 month, both groups achieved obvious improvement. Accumulated relapse rate during 1 year was 38.5% in Group 1, including 12 cases with clinical relapse and 8 patients manifesting only serological relapse; whereas there was 12.0% of relapse in Group 2, only 1 with clinical relapse and other 5 patients got serological relapse. The mean flare time in Group II was significantly longer than that in Group I. All relapsing patients in Group I were sensitive to immunosuppressants. Most patients involving more than 6 organs in Group I relapsed during 1 year. IgG4 levels of relapse cases were significantly higher than non-relapsing patients at baseline. Bile duct, lacrimal glands and lymph nodes were commonly relapsed organs in Group I.

Publication types

  • Clinical Trial
  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cyclophosphamide / administration & dosage*
  • Cyclophosphamide / therapeutic use
  • Drug Administration Schedule
  • Drug Therapy, Combination
  • Female
  • Humans
  • Immunoglobulin G4-Related Disease / drug therapy*
  • Male
  • Middle Aged
  • Prednisone / administration & dosage*
  • Prednisone / therapeutic use
  • Prospective Studies
  • Recurrence
  • Treatment Outcome

Substances

  • Cyclophosphamide
  • Prednisone