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

• Published in: Scientific reports Vol. 7; no. 1; pp. 6195 - 8
• 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: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 21.07.2017; Nature Publishing Group; Nature Portfolio
• Subjects: 692/4023/1670; 692/699/249/1313; Adult; Autoimmune diseases; Bile ducts; Cyclophosphamide; Cyclophosphamide - administration & dosage; Cyclophosphamide - therapeutic use; Drug Administration Schedule; Drug Therapy, Combination; Female; Glands; Glucocorticoids; Hospitals; Humanities and Social Sciences; Humans; Immunoglobulin G; Immunoglobulin G4; Immunoglobulin G4-Related Disease - drug therapy; Immunoglobulins; Immunosuppressive agents; Lymph nodes; Male; Medical schools; Middle Aged; multidisciplinary; Prednisone; Prednisone - administration & dosage; Prednisone - therapeutic use; Prospective Studies; Recurrence; Remission; Science; Science (multidisciplinary); Serology; Systemic diseases; Treatment Outcome
• ISSN: 2045-2322; 2045-2322
• DOI: 10.1038/s41598-017-06520-5

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.