毛细血管显微镜检查是一种潜在的用于儿童雷诺现象的结缔组织疾病的筛查方法。

Capillary microscopy is a potential screening method for connective tissue disease in children with Raynaud's phenomenon.

机构信息

Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, Groningen, The Netherlands.

Deptartment of Internal Medicine, division Vascular Medicine, University of Groningen, University Medical Centre Groningen, Groningen, the Netherlands.

出版信息

Pediatr Rheumatol Online J. 2022 Feb 8;20(1):11. doi: 10.1186/s12969-022-00671-0.

DOI:10.1186/s12969-022-00671-0

PMID:35135560

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8822798/

Abstract

BACKGROUND

Nailfold capillary microscopy (NCM) is a cornerstone in the diagnosis of Systemic Sclerosis (SSc) in adulthood. Although Raynaud's phenomenon (RP) is very common in childhood, studies on diagnostic methods to differentiate between primary RP (PRP) and secondary RP (SRP) at a young age are scarce. The aim of this study was to determine the value of NCM in differentiating between PRP and SRP in children and adolescents with RP.

METHODS

In this nested case-control study, 83 patients diagnosed with RP and having underwent NCM in childhood were retrospectively included. Based on whether they were diagnosed with a connective tissue disease (CTD) during follow-up, patients were classified as PRP or SRP. NCM was performed by a vascular technician. PRP and SRP patients were compared on demographics, NCM and serology. Variables associated with SRP were included in a multivariate logistic regression model. Predictive values were calculated for NCM, ANA positivity and the combination of NCM and ANA positivity.

RESULTS

At the time of the NCM, the mean age of the RP patients was 15.4 ± 2.3 years. Of these patients, 78.3% were classified as PRP and 21.7% as SRP at mean follow-up of 6.4 ± 3.20 years. CTDs were miscellaneous, with only one patient having developed SSc. Of the NCM parameters, only capillary loss was associated with SRP (p = 0.01). In a multivariate logistic regression model including ANA, capillary loss was not a predictor of SRP. In a model without ANAs, capillary loss was an independent predictor (OR = 3.98, CI 95% 1.22-12.99). Capillary loss had a sensitivity of 44.4% and a specificity of 84.4% for SRP. ANA combined with capillary loss had a sensitivity of 66.7% and a specificity of 85.7%.

CONCLUSION

Whereas RP in adulthood is most strongly associated with SSc, children with RP seem to be at risk for developing other CTDs with less apparent NCM abnormalities. Of all NCM findings, only capillary loss was predictive for SRP. NCM did not add to the predictive value of ANA screening. However, with a specificity of 84.4% and being non-invasive, NCM shows potential as a screening method for SRP. More research with a larger study population is required before drawing conclusions.

摘要

背景

甲襞毛细血管显微镜检查（NCM）是成人系统性硬化症（SSc）诊断的基石。尽管雷诺现象（RP）在儿童中非常常见，但关于在年轻时区分原发性 RP（PRP）和继发性 RP（SRP）的诊断方法的研究很少。本研究旨在确定 NCM 在区分儿童和青少年 RP 中的 PRP 和 SRP 的价值。

方法

在这项嵌套病例对照研究中，回顾性纳入了 83 例诊断为 RP 并在儿童时期接受过 NCM 的患者。根据他们在随访期间是否被诊断为结缔组织疾病（CTD），将患者分为 PRP 或 SRP。NCM 由血管技术员进行。比较 PRP 和 SRP 患者的人口统计学、NCM 和血清学数据。将与 SRP 相关的变量纳入多元逻辑回归模型。计算 NCM、ANA 阳性和 NCM 和 ANA 阳性联合的预测值。

结果

在 NCM 时，RP 患者的平均年龄为 15.4 ± 2.3 岁。在平均 6.4 ± 3.20 年的随访中，这些患者中有 78.3%被归类为 PRP，21.7%为 SRP。CTD 为杂项，仅有 1 例患者发生 SSc。在 NCM 参数中，仅毛细血管缺失与 SRP 相关（p = 0.01）。在不包括 ANA 的多元逻辑回归模型中，毛细血管缺失不是 SRP 的预测因素。在没有 ANAs 的模型中，毛细血管缺失是一个独立的预测因素（OR = 3.98，95%CI 1.22-12.99）。毛细血管缺失对 SRP 的敏感性为 44.4%，特异性为 84.4%。ANA 联合毛细血管缺失对 SRP 的敏感性为 66.7%，特异性为 85.7%。

结论

虽然成年期的 RP 与 SSc 关系最为密切，但 RP 儿童似乎有发展其他 CTD 的风险，其 NCM 异常不太明显。在所有 NCM 发现中，只有毛细血管缺失可预测 SRP。NCM 并未增加 ANA 筛查的预测价值。然而，NCM 的特异性为 84.4%，且为非侵入性，因此具有作为 SRP 筛查方法的潜力。需要更多具有更大研究人群的研究来得出结论。