Department of Pediatrics, Division of Rheumatology, Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Rheumatology (Oxford). 2010 Feb;49(2):373-81. doi: 10.1093/rheumatology/kep361. Epub 2009 Dec 14.
To develop and assess the psychometric properties of the Localized Scleroderma (LS) Skin Damage Index (LoSDI) and Physician Global Assessment of disease Damage (PGA-D).
Damage was defined as irreversible/persistent changes (>6 months) due to previous active disease/complications of therapy. Eight rheumatologists assessed the importance of 17 variables in formulating the PGA-D/LoSDI. LS patients were evaluated by two rheumatologists using both tools to assess their psychometric properties. LoSDI was calculated by summing three scores for cutaneous features of damage [dermal atrophy (DAT), subcutaneous atrophy (SAT) and dyspigmentation (DP)] measured at 18 anatomic sites. Patient GA of disease severity (PtGA-S), Children's Dermatology Life Quality Index (CDLQI) and PGA-D were recorded at the time of each examination.
Thirty LS patients (112 lesions) and nine patient-visit pairs (18 lesions) were included for inter- and intra-rater reliability study. LoSDI and its domains DAT, SAT, DP and PGA-D demonstrated excellent inter- and intra-rater reliability (reliability coefficients 0.86-0.99 and 0.74-0.96, respectively). LoSDI correlated moderately with PGA-D and poorly with PtGA-S and CDLQI. PGA-D correlated moderately with PtGA-S, but poorly with CDLQI.
To complete the LS Cutaneous Assessment Tool (LoSCAT), we developed and evaluated the psychometric properties of the LoSDI and PGA-D in addition to the LS Skin Severity Index (LoSSI). These instruments will facilitate evaluation of LS patients for individual patient management and clinical trials. LoSDI and PGA-D demonstrated excellent reliability and high validity. LoSCAT provides an improved understanding of LS natural history. Further study in a larger group of patients is needed to confirm these preliminary findings.
开发并评估局限性硬皮病（LS）皮肤损伤指数（LoSDI）和医师整体疾病损伤评估（PGA-D）的心理测量特性。
损伤定义为先前活动性疾病/治疗并发症引起的不可逆/持续性变化（>6 个月）。8 位风湿病学家评估了 17 个变量在制定 PGA-D/LoSDI 中的重要性。两位风湿病学家使用这两种工具评估 LS 患者，以评估其心理测量特性。LoSDI 通过对 18 个解剖部位测量的三种皮肤损伤特征（真皮萎缩（DAT）、皮下萎缩（SAT）和色素沉着异常（DP））的三个得分进行求和来计算。在每次检查时记录患者疾病严重程度的总体评估（PtGA-S）、儿童皮肤病生活质量指数（CDLQI）和 PGA-D。
30 名 LS 患者（112 处病变）和 9 对患者就诊（18 处病变）被纳入进行观察者间和观察者内可靠性研究。LoSDI 及其域 DAT、SAT、DP 和 PGA-D 表现出极好的观察者间和观察者内可靠性（可靠性系数分别为 0.86-0.99 和 0.74-0.96）。LoSDI 与 PGA-D 中度相关，与 PtGA-S 和 CDLQI 相关性差。PGA-D 与 PtGA-S 中度相关，但与 CDLQI 相关性差。
为了完成 LS 皮肤评估工具（LoSCAT），我们开发并评估了 LoSDI 和 PGA-D 的心理测量特性，以及 LS 皮肤严重程度指数（LoSSI）。这些工具将有助于对 LS 患者进行个体患者管理和临床试验的评估。LoSDI 和 PGA-D 表现出极好的可靠性和高度的有效性。LoSCAT 提供了对 LS 自然史的更好理解。需要在更大的患者群体中进行进一步研究，以确认这些初步发现。
