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

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 gradual...

Full description

Saved in:
Bibliographic Details
Published in:Scientific reports 2017-07, Vol.7 (1), p.6195-8, Article 6195
Main Authors: Yunyun, Fei, Yu, Chen, Panpan, Zhang, Hua, Chen, Di, Wu, Lidan, Zhao, Linyi, Peng, Li, Wang, Qingjun, Wu, Xuan, Zhang, Yan, Zhao, Xiaofeng, Zeng, Fengchun, Zhang, Wen, Zhang
Format: Article
Language:English
Subjects:
692/4023/1670
692/699/249/1313
Autoimmune diseases
Bile ducts
Cyclophosphamide
Glands
Glucocorticoids
Hospitals
Humanities and Social Sciences
Immunoglobulin G
Immunoglobulin G4
Immunoglobulins
Immunosuppressive agents
Lymph nodes
Medical schools
multidisciplinary
Prednisone
Remission
Science
Science (multidisciplinary)
Serology
Systemic diseases
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2045-2322
2045-2322
DOI:10.1038/s41598-017-06520-5