Division of Pediatric Rheumatology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Box 50, Chicago, IL, 60611, USA.
Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Pediatr Rheumatol Online J. 2023 Oct 13;21(1):118. doi: 10.1186/s12969-023-00903-x.
We lack a reliable indicator of disease activity in Juvenile Dermatomyositis (JDM), a rare disease. The goal of this study is to identify the association of nailfold capillary End Row Loop (ERL) loss with disease damage in children with newly diagnosed, untreated JDM.
We enrolled 140 untreated JDM and 46 age, race and sex matched healthy controls, ages 2-17. We selected items from the Juvenile Myositis Registry for analysis. Variables include average ERL density of 8 fingers, average capillary pattern, hemorrhages, and clinical and laboratory correlates. Laboratory data includes Myositis Specific Antibodies (MSA), disease activity scores (DAS), Childhood Myositis Assessment Scale (CMAS), and standard clinical serologic data. The reduced mean ERL density is 5.1 ± 1.5/mm for untreated JDM vs 7.9 ± 0.9/mm for healthy controls, p < 0.0001, and is associated with DAS-skin, r = -0.27 p = 0.014, which did not change within the age range tested. Untreated JDM with MSA Tif-1-γ had the lowest ERL density, (p = 0.037); their ERL patterns were primarily "open" and the presence of hemorrhages in the nailfold matrix was associated with dysphagia (p = 0.004).
Decreased JDM ERL density is associated with increased clinical symptoms; nailfold hemorrhages are associated with dysphagia. Duration of untreated disease symptoms and MSA, modify NFC shape. We speculate nailfold characteristics are useful indicators of disease activity in children with JDM before start of therapy.
我们缺乏幼年特发性皮肌炎（JDM）这种罕见疾病活动的可靠指标。本研究旨在确定新诊断未治疗 JDM 患儿指甲皱襞毛细血管终末环（ERL）缺失与疾病损害的相关性。
我们纳入了 140 例未经治疗的 JDM 患儿和 46 例年龄、种族和性别匹配的健康对照者，年龄 2-17 岁。我们从幼年特发性肌炎登记处选择项目进行分析。变量包括 8 个手指的平均 ERL 密度、平均毛细血管模式、出血以及临床和实验室相关因素。实验室数据包括肌炎特异性抗体（MSA）、疾病活动评分（DAS）、儿童肌炎评估量表（CMAS）和标准临床血清学数据。未经治疗的 JDM 的平均 ERL 密度降低，为 5.1±1.5/mm，而健康对照组为 7.9±0.9/mm，p<0.0001，与 DAS-皮肤相关，r=-0.27，p=0.014，在测试的年龄范围内没有变化。具有 Tif-1-γ MSA 的未经治疗的 JDM 患者的 ERL 密度最低（p=0.037）；他们的 ERL 模式主要是“开放”，指甲皱襞基质中的出血与吞咽困难相关（p=0.004）。
JDM ERL 密度降低与临床症状增加相关；指甲皱襞出血与吞咽困难相关。未治疗疾病症状的持续时间和 MSA 改变了 NFC 的形状。我们推测，指甲特征是儿童 JDM 开始治疗前疾病活动的有用指标。
