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

Efficacy of Cyclophosphamide treatment for immunoglobulin G4-related disease with addition of glucocorticoids.
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Yunyun F, Yu C, Panpan Z, Hua C, Di W, Lidan Z, Linyi P, Li W, Qingjun W, Xuan Z, Yan Z, Xiaofeng Z, Fengchun Z, Wen Z,


Yunyun F, Yu C, Panpan Z, Hua C, Di W, Lidan Z, Linyi P, Li W, Qingjun W, Xuan Z, Yan Z, Xiaofeng Z, Fengchun Z, Wen Z, (click to view)

Yunyun F, Yu C, Panpan Z, Hua C, Di W, Lidan Z, Linyi P, Li W, Qingjun W, Xuan Z, Yan Z, Xiaofeng Z, Fengchun Z, Wen Z,

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Scientific reports 2017 07 217(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.

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