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红细胞增多性肢痛症:皮质类固醇反应亚组的鉴定。

Erythromelalgia: Identification of a corticosteroid-responsive subset.

机构信息

Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota.

出版信息

J Am Acad Dermatol. 2017 Mar;76(3):506-511.e1. doi: 10.1016/j.jaad.2016.08.048. Epub 2016 Oct 26.

DOI:10.1016/j.jaad.2016.08.048
PMID:28413058
Abstract

BACKGROUND

Corticosteroids (CS) may benefit certain patients with erythromelalgia.

OBJECTIVES

Our objective was to determine clinical predictors of corticosteroid-responsive erythromelalgia.

METHODS

Patients with erythromelalgia who received CS were identified and stratified into corticosteroid nonresponders (NRs), partial corticosteroid responders (PSRs), complete corticosteroid responders (CSRs), and steroid responders (SRs = PSRs + CSRs). In the study variable analysis, P < .05 was considered statistically significant.

RESULTS

The median (interquartile range) age of the 31-patient cohort was 47 years (26-57 years), and 22 (71%) were female. Fourteen (45%) were NRs, 17 (55%) SRs, 8 (26%) PSRs, and 9 (29%) CSRs. A subacute temporal profile to disease zenith (<21 days) was described in 15 (48%) patients, of whom 13 (87%) were SRs (P = .003; odds ratio [OR] = 0.069 [95% confidence interval {CI}, 0.011-0.431]). Six (67%) CSRs reported a disease precipitant (eg, surgery, trauma, or infection; P = .007; OR = 12.667 [95% CI, 2-80.142]). SR patients received CS sooner than NR at 3 (3-12) versus 24 (17-45) months (P = .003). A high-dose CS trial (≥200 mg prednisone cumulatively) was administered to 17 (55%) patients, of whom 13 (76%) were SRs (P = .012; OR = 8.125 [95% CI, 1.612-40.752]).

LIMITATIONS

This was a retrospective case series.

CONCLUSION

An infectious, traumatic, or surgical precipitant and subacute presentation may portend CR erythromelalgia. A transient "golden window" where CS intervention is useful may exist before irreversible nociceptive remodeling and central sensitization occurs.

摘要

背景

皮质类固醇(CS)可能对某些红斑性肢痛症患者有益。

目的

我们的目的是确定皮质类固醇反应性红斑性肢痛症的临床预测因子。

方法

确定接受 CS 治疗的红斑性肢痛症患者,并将其分为皮质类固醇无反应者(NRs)、部分皮质类固醇反应者(PSRs)、完全皮质类固醇反应者(CSRs)和类固醇反应者(SRs=PSRs+CSRs)。在研究变量分析中,P<.05 被认为具有统计学意义。

结果

31 例患者队列的中位(四分位间距)年龄为 47 岁(26-57 岁),其中 22 例(71%)为女性。14 例(45%)为 NRs,17 例(55%)为 SRs,8 例(26%)为 PSRs,9 例(29%)为 CSRs。15 例(48%)患者疾病高峰呈亚急性时间模式(<21 天),其中 13 例(87%)为 SRs(P=.003;优势比[OR]=0.069[95%置信区间{CI},0.011-0.431])。6 例(67%)CSRs 报告有疾病诱发因素(例如手术、创伤或感染;P=.007;OR=12.667[95%CI,2-80.142])。SR 患者接受 CS 的时间早于 NR 患者,分别为 3(3-12)个月和 24(17-45)个月(P=.003)。17 例(55%)患者接受了高剂量 CS 试验(累积>200mg 泼尼松),其中 13 例(76%)为 SRs(P=.012;OR=8.125[95%CI,1.612-40.752])。

局限性

这是一项回顾性病例系列研究。

结论

感染、创伤或手术诱发因素和亚急性表现可能预示着 CR 红斑性肢痛症。在不可逆的伤害性重塑和中枢敏化发生之前,可能存在 CS 干预有用的短暂“黄金窗口”。

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