Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids

Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids
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DOI:
10.1016/j.jaad.2013.05.016
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发表时间:
2013-10-01
影响因子:
13.8
通讯作者:
Joly, Pascal
Joly, Pascal
中科院分区:
医学1区
文献类型:
--
作者:
Almugairen, Naif;Hospital, Valerie;Joly, Pascal

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背景:很少有研究根据共识声明的定义评估天疱疮治疗。完全缓解停止治疗 (CRoffT) 的预后因素仍不清楚。 目的:我们试图评估接受不同方案治疗的天疱疮患者的 CRoffT 率。 方法:一项回顾性多中心研究总共纳入了 134 名天疱疮患者。主要终点是 CRoffT 率。采用单变量和多变量分析确定 CRoffT 的预后因素。结果:纳入 80 例寻常型天疱疮患者、47 例落叶性天疱疮患者和 7 例副肿瘤性天疱疮患者。平均年龄为 60 +/- 18 岁。患者接受中等剂量(= 1 mg/kg/d)(n = 59)泼尼松治疗,或不接受全身皮质类固醇治疗(n = 43)。平均随访时间为 77 +/- 64 个月。总共有 68 名患者 (50.7%) 在平均治疗持续时间 36 +/- 39 个月后达到 CRoffT(95% 置信区间 42.3%-59.2%),其中包括 80 名寻常型天疱疮患者中的 47 名(58.7%)和 47 名落叶型天疱疮患者中的 21 名(44.7%)。 CRoffT 的主要预后因素是初始粘膜受累(风险比 2.2;95% 置信区间 1.05-4.58;P = .036)和较年轻的年龄(
Background: Few studies have evaluated pemphigus treatments according to the definitions of the consensus statement. Prognostic factors for complete remission off therapy (CRoffT) remain unknown.Objective: We sought to assess the rate of CRoffT in patients with pemphigus treated with different regimens.Methods: In all, 134 patients with pemphigus were included in a retrospective, multicenter study. Primary end point was the rate of CRoffT. Prognostic factors for CRoffT were determined using univariate and multivariate analyses.Results: Eighty patients with pemphigus vulgaris, 47 with pemphigus foliaceus, and 7 with paraneoplastic pemphigus were included. Mean age was 60 +/- 18 years. Patients were treated either with medium (= 1 mg/kg/d) (n = 59) doses of prednisone, or without systemic corticosteroids (n = 43). Mean follow-up was 77 +/- 64 months. In all, 68 patients (50.7%) achieved CRoffT (95% confidence interval 42.3%-59.2%) after a mean treatment duration of 36 +/- 39 months, including 47 of 80 patients with pemphigus vulgaris (58.7%) and 21 of 47 with pemphigus foliaceus (44.7%). Main prognostic factors for CRoffT were initial mucosal involvement (hazard ratio 2.2; 95% confidence interval 1.05-4.58; P = .036) and younger age (