University of Texas Southwestern Medical Center, Dallas.
Arthritis Rheumatol. 2014 Dec;66(12):3496-504. doi: 10.1002/art.38853.
Few studies have examined outcomes in adults with pediatric-onset morphea. The objective of the present study was to compare clinical outcomes and health-related quality of life (HRQOL) in adults with onset of morphea in childhood to those in patients with adult onset of morphea.
Participants in the study were drawn from the Morphea in Adults and Children cohort and included 68 adults with pediatric-onset morphea and 234 patients with adult-onset morphea. Outcome measures included the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), physical examination findings, and HRQOL questionnaires.
Adults with pediatric-onset morphea were younger, had longer disease duration, and were more likely to have the linear subtype of morphea. Patients with pediatric-onset disease were less likely to have active disease. Among patients with active disease, those with pediatric-onset morphea had less disease activity as measured by the LoSCAT. Patients with pediatric-onset disease had higher severity of disease damage when measured by the physician's global assessment of damage, but had similar levels of disease damage when measured by the Localized Scleroderma Skin Damage Index. Patients with pediatric-onset disease had more favorable HRQOL scores for all measures, all of which were statistically significantly different from those in patients with adult-onset morphea.
Adults with pediatric-onset morphea differ from patients with adult-onset disease with respect to disease subtype, severity of disease activity and damage, and levels of HRQOL.
鲜有研究调查过儿童期发病的硬斑病患者的结局。本研究的目的是比较儿童期发病的硬斑病患者与成人期发病的硬斑病患者的临床结局和健康相关生活质量(HRQOL)。
该研究的参与者来自硬斑病成人和儿童队列,包括 68 例儿童期发病的硬斑病成年患者和 234 例成人期发病的硬斑病患者。结局测量包括局限性硬皮病皮肤评估工具(LoSCAT)、体格检查结果和 HRQOL 问卷。
儿童期发病的硬斑病成年患者更年轻,疾病持续时间更长,且更可能患有线状硬斑病。儿童期发病的患者更可能处于疾病活动期。在患有活动性疾病的患者中,儿童期发病的硬斑病患者的 LoSCAT 测量的疾病活动度较低。通过医生整体评估损害来测量时,儿童期发病的硬斑病患者的疾病损害严重程度更高,但通过局限性硬皮病皮肤损害指数来测量时,疾病损害程度相似。在所有测量指标中,儿童期发病的硬斑病患者的 HRQOL 评分都更好,所有这些指标均与成人期发病的硬斑病患者存在统计学差异。
与成人期发病的硬斑病患者相比,儿童期发病的硬斑病患者在疾病亚型、疾病活动度和损害严重程度以及 HRQOL 水平方面存在差异。