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原发性雷诺现象与结缔组织病继发的临床相关差异。

Clinically relevant differences between primary Raynaud's phenomenon and secondary to connective tissue disease.

机构信息

Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom.

Department of Internal Medicine, Division of Rheumatology, Scleroderma Program, University of Michigan, Ann Arbor, USA; Department of Biostatistics, School of Public Health, University of Michigan, Ann Arbor, MI, USA.

出版信息

Semin Arthritis Rheum. 2024 Oct;68:152521. doi: 10.1016/j.semarthrit.2024.152521. Epub 2024 Jul 18.

DOI:10.1016/j.semarthrit.2024.152521
PMID:39089171
Abstract

OBJECTIVES

Raynaud's phenomenon (RP) is a symptom complex associated with digital vascular compromise. Our aim was to examine for clinically relevant differences between primary RP (PRP) and secondary RP (SRP) to connective tissue disease.

METHODS

We report cross-sectional results from the Patient Survey of experiences of Raynaud's Phenomenon (PASRAP), which aimed to explore the broad-ranging impact of RP. The survey was widely distributed online including via social medial. Participation was voluntary and responses were anonymous.

RESULTS

1229 respondents completed PASRAP with self-reported RP: PRP 218 (17.7 %) and SRP 1011 (82.3 %) of which 903 (92.9 %) Systemic Sclerosis. The mean (SD) age was significantly lower in respondents with PRP (41.7 [11.8] vs 54.2 [12.4] years, P<0.0001). During attacks, more subjects with SRP reported cyanotic colour changes (92.2 % vs 86.5 %, P=0.0089). Patients with PRP experienced more pain (72.1 % vs 55.9 %, P<0.0001), numbness (80.3 % vs 69.4 %, P=0.0016), stinging/throbbing (93.4 % vs 80.8 %, P<0.0001), and tingling (84.0 % vs 77.5 %, P=0.0345). Only half of respondents' symptoms were adequately controlled by their current medication(s), more commonly in SRP (55.2 % vs 45.2 %, P=0.0084). There were important differences in the triggers, number, and seasonal variation of RP attacks.

CONCLUSION

There are clinically relevant differences between PRP and SRP concerning the multifaceted lived patient experience of RP. Neurosensory symptoms are more common in PRP. Patients with SRP are older and present with more colour changes, overrepresented by cyanosis, and with less complete resolution of symptoms between attacks. These data provide novel insights for future RP clinical trial design.

摘要

目的

雷诺现象(RP)是一种与数字血管损伤相关的症状复杂。我们的目的是检查原发性 RP(PRP)和与结缔组织疾病相关的继发性 RP(SRP)之间是否存在临床相关差异。

方法

我们报告了患者对雷诺现象体验的问卷调查(PASRAP)的横断面结果,该调查旨在探索 RP 的广泛影响。该调查在网上广泛发布,包括社交媒体。参与者是自愿的,答复是匿名的。

结果

1229 名受访者完成了 PASRAP,报告有 RP:PRP 218 例(17.7%),SRP 1011 例(82.3%),其中 903 例(92.9%)为系统性硬化症。PRP 患者的平均(SD)年龄明显较低(41.7[11.8]岁 vs 54.2[12.4]岁,P<0.0001)。在发作期间,更多的 SRP 患者报告出现蓝紫色变色(92.2% vs 86.5%,P=0.0089)。PRP 患者经历更多的疼痛(72.1% vs 55.9%,P<0.0001)、麻木(80.3% vs 69.4%,P=0.0016)、刺痛/搏动(93.4% vs 80.8%,P<0.0001)和刺痛(84.0% vs 77.5%,P=0.0345)。只有一半的受访者的症状通过当前药物得到充分控制,SRP 患者中更为常见(55.2% vs 45.2%,P=0.0084)。RP 发作的诱因、发作次数和季节性变化存在重要差异。

结论

PRP 和 SRP 在 RP 的多方面患者体验方面存在临床相关差异。PRP 中更常见神经感觉症状。SRP 患者年龄较大,出现更多颜色变化,更常见的是发绀,且发作之间症状缓解不完全。这些数据为未来 RP 临床试验设计提供了新的见解。

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