Children's Hospital of Pittsburgh of Univeristy of Pittsburgh Medical Center, University of Pittsburgh Scleroderma Center, Pittsburgh, PA, U.S.A.
Department of Physical Medicine and Rehabilitation, School of Medicine, University of Pittsburgh, Pittsburgh, PA, U.S.A.
Br J Dermatol. 2019 May;180(5):1183-1189. doi: 10.1111/bjd.17312. Epub 2018 Dec 5.
Localized scleroderma (LS) is an autoimmune condition of the skin and underlying tissue. Active or recurring disease can lead to cumulative tissue damage, especially in paediatric-onset disease.
To highlight the rate of relapse of LS activity in a cohort of paediatric patients and to evaluate for potential clinical and laboratory predictors of disease relapse.
Clinical and laboratory data were gathered prospectively. Patients were categorized as experiencing relapse or not, and clinical and laboratory parameters were compared. A logistic regression was fit to predict odds of relapse while controlling for multiple predictors. A subgroup of patients was also evaluated to determine the average time from treatment completion to relapse.
Seventy-seven patients were followed for the identified study duration of > 2 years and had achieved disease remission, with 35 (45%) experiencing LS relapse. Patients who were older at disease onset, antinuclear antibody (ANA) positive and without an extracutaneous manifestation (ECM) were more likely to relapse. All three variables remained significant in the multivariable logistic regression model. Results of the subgroup mirrored the larger sample. The average time between treatment completion and relapse was 21 months.
Assessment of patients with LS experiencing a relapse of disease activity has shown older age of initial LS onset and ANA positivity to be potential markers for risk of relapse. Patients meeting these parameters may require greater clinical vigilance. The presence of one or more ECM may be protective. Clinicians treating patients with LS should provide significant long-term follow-up to monitor for relapse.
局限性硬皮病（LS）是一种皮肤和皮下组织的自身免疫性疾病。活动期或复发性疾病可导致组织损伤的累积，尤其是在儿科发病的情况下。
突出儿科患者 LS 活动复发的比率，并评估疾病复发的潜在临床和实验室预测因素。
前瞻性收集临床和实验室数据。将患者分为复发组和未复发组，并比较临床和实验室参数。拟合逻辑回归以预测在控制多个预测因素的情况下复发的可能性。还对一组亚组患者进行评估，以确定从治疗完成到复发的平均时间。
77 例患者的研究随访时间超过 2 年，并达到疾病缓解，其中 35 例（45%）出现 LS 复发。发病时年龄较大、抗核抗体（ANA）阳性且无皮肤外表现（ECM）的患者更有可能复发。这三个变量在多变量逻辑回归模型中仍然具有显著性。亚组的结果与更大的样本相符。治疗完成与复发之间的平均时间为 21 个月。
对 LS 患者疾病活动复发的评估表明，初始 LS 发病年龄较大和 ANA 阳性可能是复发风险的潜在标志物。符合这些参数的患者可能需要更密切的临床监测。存在一个或多个 ECM 可能具有保护作用。治疗 LS 患者的临床医生应提供显著的长期随访，以监测复发。
