Revealing Microvascular Involvement in Pediatric Localized Scleroderma Through Nailfold Capillaroscopy.

IF 2.1 4区 医学 Q2 PEDIATRICS
Sema Nur Taşkın, Şeyda Doğantan, Esra Esen, Sümeyra Özdemir Çiçek, Ayşenur Paç Kısaarslan, Muammer Hakan Poyrazoğlu
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Abstract

Background/Objectives: Juvenile localized scleroderma (jLoS) is a chronic inflammatory disorder with skin and subcutaneous tissue involvement. Microvascular alterations are thought to contribute to its pathogenesis. This study aimed to investigate microvascular alterations in children with jLoS using nailfold capillaroscopy (NFC) and to compare the capillaroscopic findings between patients and healthy controls. Methods: A total of 13 children diagnosed with jLoS and 16 age- and sex-matched healthy controls were enrolled. Capillaroscopic assessment included capillary density, tortuosity, dilatation, disorganization, branching, and neoangiogenesis. Dilated and giant capillaries, hemorrhages, avascular areas, and capillary loss were evaluated. The Microangiopathy Evaluation Score (MES) was used to semi-quantitatively assess capillary loss, disorganization, and ramifications. Disease activity and damage were evaluated using the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Damage Index (LoSDI), respectively. Functional status was measured via the 6 min walk test (6MWT). Results: Plaque morphea was the most common subtype (61.5%), and antinuclear antibody (ANA) positivity was present in 53.8% of patients. Compared to controls, jLoS patients exhibited significantly more frequent capillaroscopic abnormalities, including increased tortuosity, crossing, dilatation, and neoangiogenesis (p < 0.05). Capillary density, length, arterial limb width, apical loop width, and disorganization scores were significantly higher, while intercapillary distance was lower in jLoS patients (p < 0.05). No avascular areas or giant capillaries were observed. MESs were similar between groups. Conclusions: NFC revealed significant microvascular alterations in jLoS patients, supporting its utility as a non-invasive tool for early vascular assessment in localized scleroderma.

Abstract Image

通过甲襞毛细血管镜观察小儿局限性硬皮病的微血管病变。
背景/目的:青少年局限性硬皮病(jLoS)是一种累及皮肤和皮下组织的慢性炎症性疾病。微血管的改变被认为是其发病的原因之一。本研究旨在利用甲襞毛细血管镜(NFC)研究jLoS患儿的微血管改变,并比较患者和健康对照组的毛细血管镜检查结果。方法:共纳入13名诊断为jLoS的儿童和16名年龄和性别匹配的健康对照。毛细血管镜评估包括毛细血管密度、扭曲、扩张、紊乱、分支和新生血管生成。评估扩张和巨大的毛细血管,出血,无血管区域和毛细血管损失。微血管病评估评分(MES)用于半定量评估毛细血管损失、紊乱和分支。分别采用改良的局部硬皮病皮肤严重程度指数(mLoSSI)和局部硬皮病损伤指数(LoSDI)评估疾病活动性和损伤程度。通过6分钟步行试验(6MWT)测量功能状态。结果:斑块斑斑是最常见的亚型(61.5%),抗核抗体(ANA)阳性占53.8%。与对照组相比,jLoS患者表现出更频繁的毛细血管镜异常,包括扭曲、交叉、扩张和新生血管生成增加(p < 0.05)。jLoS患者的毛细血管密度、长度、动脉肢宽、根尖袢宽度和紊乱评分均显著高于jLoS患者,而毛细血管间距则显著低于jLoS患者(p < 0.05)。未见无血管区或巨毛细血管。两组间的MESs相似。结论:NFC显示了jLoS患者明显的微血管改变,支持其作为局限性硬皮病早期血管评估的非侵入性工具的实用性。
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来源期刊
Children-Basel
Children-Basel PEDIATRICS-
CiteScore
2.70
自引率
16.70%
发文量
1735
审稿时长
6 weeks
期刊介绍: Children is an international, open access journal dedicated to a streamlined, yet scientifically rigorous, dissemination of peer-reviewed science related to childhood health and disease in developed and developing countries. The publication focuses on sharing clinical, epidemiological and translational science relevant to children’s health. Moreover, the primary goals of the publication are to highlight under‑represented pediatric disciplines, to emphasize interdisciplinary research and to disseminate advances in knowledge in global child health. In addition to original research, the journal publishes expert editorials and commentaries, clinical case reports, and insightful communications reflecting the latest developments in pediatric medicine. By publishing meritorious articles as soon as the editorial review process is completed, rather than at predefined intervals, Children also permits rapid open access sharing of new information, allowing us to reach the broadest audience in the most expedient fashion.
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