Background

Glucocorticoid (GC)-dependent, colchicine-resistant idiopathic recurrent pericarditis (IRP) remains a clinical challenge. We assessed for the first time the efficacy and safety of hydroxychloroquine (HCQ) in IRP.

Methods and results

This is a post hoc analysis of prospectively collected data of 15 patients with refractory to standard therapy (colchicine plus either GC or anakinra) IRP (≥3 recurrences, disease duration ≥12 months and inability to wean off treatment) treated with HCQ (400 mg/day). These patients were matched 1:1 for age, sex, and treatment type to IRP patients receiving standard-of-care treatment (control group, n = 15). Pericarditis recurrence, the time to 1st flare, the % of patients able to achieve a ≥50% reduction of baseline GC dose and the % reduction of GC dose, were compared between groups. HCQ did not reduce pericarditis recurrence risk as almost all patients (n = 29) but one in the HCQ group (14/15) relapsed during follow-up. However, HCQ treatment was associated with an increased median time of flare-free survival (increase by 4 weeks compared to controls) and reduced hazard ratio for flare in survival analysis (HR = 0.36, 95% CI 0.16–0.77, p = 0.009). HCQ was also associated with a higher proportion of patients obtaining a ≥50% dose reduction of GCs (33.3% vs. 0% in the control group, p = 0.037) and reduced GC dose (HCQ: −43.5% vs. control: −4.5%, p < 0.001). No differences in CRP levels at flare was detected (p = 0.615).

Conclusions

In this prospective study, HCQ depicted a GC-sparing effect and an increased flare-free survival period in patients with colchicine resistant GC-dependent IRP.

中文翻译：

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羟氯喹对秋水仙碱耐药的糖皮质激素依赖型特发性复发性心包炎的研究：一项前瞻性观察性前瞻性研究。

背景

糖皮质激素（GC）依赖性，秋水仙碱耐药的特发性复发性心包炎（IRP）仍然是临床挑战。我们首次评估了羟氯喹（IRQ）的疗效和安全性。

方法与结果

这是对15例接受HCQ（400 mg治疗）的标准治疗（秋水仙碱加GC或anakinra），IRP（≥3复发，疾病持续时间≥12个月且无法戒断治疗）难治的患者的前瞻性收集数据的事后分析。 /天）。这些患者的年龄，性别和治疗类型与接受护理标准治疗的IRP患者按1：1匹配（对照组，n = 15）。比较两组之间的心包炎复发，第一次发作的时间，能够降低基线GC剂量≥50％的患者百分比和降低GC剂量的百分比。HCQ不能降低心包炎复发的风险，因为几乎所有患者（n = 29），但HCQ组中的一名患者（14/15）在随访期间复发。然而，HCQ治疗与无火炬存活的中位数时间增加（与对照组相比增加了4周）和生存分析中火炬的危险比降低有关（HR = 0.36，95％CI 0.16-0.77，p = 0.009）。HCQ还与更高比例的患者获得≥5％的GC降低剂量相关（33.3％比对照组的0％，p = 0.037）和降低的GC剂量（HCQ：−43.5％vs.对照：– 4.5％，p <0.001）。在爆发时未检测到CRP水平的差异（p = 0.615）。

结论

在这项前瞻性研究中，HCQ描述了具有秋水仙碱抗GC依赖性IRP的患者的GC保留效应和无眩晕生存期增加。