Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study
Clinical outcomes and predictive relapse factors of IgG4-related disease following treatment: a long-term cohort study
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IgG4相关疾病治疗后的临床结果和预测复发因素：一项长期队列研究
DOI：
10.1111/joim.12942
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发表时间：
2019-11-01
影响因子：
11.1
通讯作者：
Zhang, W.
中科院分区：
文献类型：
--
作者：
Peng, Y.;Li, J. Q.;Zhang, W.
目的探讨IgG 4相关疾病（IgG 4-RD）复发的预测因素，并观察IgG 4-RD患者的长期临床转归。方法对122例新诊断的IgG 4-RD患者进行糖皮质激素（GC）单药治疗或GC与免疫抑制剂联合治疗，并随访至少3年。记录临床复发情况、疗效及不良反应。结果12、24和36个月时的累积复发率分别为10.66%、22.95%和27.87%。完全停药是疾病复发的独立危险因素。较高的血清IgG 4浓度、累及更多器官、较高的IgG 4 RI评分和基线时嗜酸性粒细胞升高与疾病复发密切相关。随访期间血清IgG 4浓度的再升高和低GC维持剂量与临床复发显著相关。维持期单药治疗的GC剂量应大于6.25 mg/d（-1），联合免疫抑制剂可降低GC剂量。对血清IgG 4水平再升高的患者加用GC或免疫抑制剂可预防后期疾病复发。长期随访未发现严重并发症。结论在激素减量和维持阶段，GC联合免疫抑制剂比单用GC更有效。血清IgG 4水平升高、累及器官多、IgG 4 RI评分升高、过敏史、基线时嗜酸性粒细胞升高、随访时血清IgG 4水平再升高和GC维持剂量降低可能预示复发。
Objectives The aim of this study was to investigate the predictive factors for relapse of IgG4-related disease (IgG4-RD) and observe the long-term clinical outcomes in patients with IgG4-RD. Methods We included in the present analysis 122 patients who were newly diagnosed with IgG4-RD, treated with glucocorticoid (GC) monotherapy or GC and immunosuppressant combination therapy, and followed for at least 3 years. Clinical relapse, response and side effects were recorded. Results The cumulative relapse rates of patients in this study were 10.66%, 22.95% and 27.87% at 12, 24 and 36 months, respectively. Complete drug withdrawal was an independent risk factor for disease relapse. Higher serum IgG4 concentrations, involvement of more organs, higher IgG4 RI scores and elevation of eosinophils at baseline were closely associated with disease relapse. Re-elevation of serum IgG4 concentrations and low GC maintenance dosage during the follow-up period were significantly associated with clinical relapse. The GC dosage should be more than 6.25 mg day(-1) as monotherapy during the maintenance stage; moreover, combining with immunosuppressants can reduce the GC dosage. Adding GC or immunosuppressants for patients with re-elevation of serum IgG4 levels could prevent later disease relapse. No serious complications were noted during long-term follow-up. Conclusions The combination of GC with immunosuppressants was more effective than GC monotherapy during the steroid tapering and maintenance stages. Higher serum IgG4 levels, involvement of more organs, higher IgG4 RI scores, history of allergy, eosinophil elevation at baseline, re-elevation of serum IgG4 levels and lower GC maintenance dosage at follow-up might be predictive of relapse.