Objectives The aim of this study was to validate the Patient self-Assessment of Skin Thickness in Upper Limb questionnaire (PASTUL) in systemic sclerosis (SSc) and assess impact of skin involvement on health-related quality of life (HRQoL). Methods Participants were included in four UK centres. PASTUL specifies a grading of skin at 8 sites corresponding to the modified Rodnan Skin Score (mRSS). Construct validity was assessed by comparing PASTUL scores with mRSS. HRQoL was evaluated with EQ5D5L and Leeds SSc HRQoL questionnaires. Additionally, correlation between PASTUL and Scleroderma Skin Patient reported Outcome (SSPRO) was explored. Follow-up was 12 months. Results In total, 196 participants were included, mean age was 56.4 years (SD 13.9), 80.6% female (n = 158), mean disease duration 11.9 years (SD 9.9), 110 (56.1%) had limited cutaneous (lcSSc) and 81 (41.3%) diffuse cutaneous SSc (dcSSc). PASTUL and upper limb mRSS were well correlated at baseline, 6 and 12 months (ICC = 0.67, 0.78 and 0.62, p< 0.001). Test-retest reliability was good (ICC = 0.83, p< 0.001). There was a stronger correlation between PASTUL and upper limb mRSS in dcSSc compared with lcSSc (0.69 vs 0.51, p< 0.001). In participants with early disease (< 4 years) PASTUL was moderately correlated with HRQoL (r = 0.53, p< 0.001), correlations were weaker in the whole group. Mean time to do the PASTUL self-assessment was 5.0 min (SD 3.7). Conclusion PASTUL is a feasible outcome tool that adds to assessments as SSPRO. Skin thickening is correlated with HRQoL, particularly in early disease.

中文翻译：

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系统性硬化症患者自我报告的皮肤严重程度和生活质量：PASTUL 的多中心验证


目的 本研究的目的是验证系统性硬化症 （SSc） 上肢皮肤厚度患者自我评估问卷 （PASTUL），并评估皮肤受累对健康相关生活质量 （HRQoL） 的影响。方法 参与者被纳入四个英国中心。PASTUL 规定了对应于改良 Rodnan 皮肤评分 （mRSS） 的 8 个部位的皮肤分级。通过将 PASTUL 评分与 mRSS 进行比较来评估构建体效度。使用 EQ5D5L 和 Leeds SSc HRQoL 问卷评估 HRQoL。此外，探讨了 PASTUL 与硬皮病患者报告结果 （SSPRO） 之间的相关性。随访 12 个月。结果 共纳入 196 名参与者，平均年龄 56.4 岁 （SD 13.9），80.6% 女性 （n = 158），平均病程 11.9 年 （SD 9.9），110 名 （56.1%） 患有皮肤局限 （lcSSc） 和 81 名 （41.3%） 弥漫性皮肤 SSc （dcSSc）。PASTEUL 和上肢 mRSS 在基线、 6 个月和 12 个月时具有良好的相关性 （ICC = 0.67 、 0.78 和 0.62，p< 0.001）。重测信度良好 （ICC = 0.83，p< 0.001）。与 lcSSc 相比，PASTUL 与 dcSSc 中上肢 mRSS 之间的相关性更强 （0.69 vs 0.51，p< 0.001）。在早期疾病参与者 （< 4 岁） 中，PASTUL 与 HRQoL 中度相关 （r = 0.53，p< 0.001），整个组的相关性较弱。进行 PASTUL 自我评估的平均时间为 5.0 分钟 （SD 3.7）。结论 PASTUL 是一种可行的结果工具，可以作为 SSPRO 添加到评估中。皮肤增厚与 HRQoL 相关，尤其是在疾病早期。