Objectives The aim of this study was to validate the Patient self-Assessment of Skin Thickness in Upper Limb (PASTUL) questionnaire in 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 eight sites corresponding to the modified Rodnan Skin Score (mRSS). Construct validity was assessed by comparing PASTUL scores with mRSS. HRQoL was evaluated with EuroQoL 5 dimension 5 levels (EQ5D5L) and Leeds SSc QoL 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 (s.d. 13.9), 80.6% female (n = 158), mean disease duration 11.9 years (s.d. 9.9), 110 (56.1%) had lcSSc and 81 (41.3%) dcSSc. PASTUL and upper limb mRSS were well correlated at baseline, 6 and 12 months [intraclass correlation coefficients (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 (s.d. 3.7). Conclusion PASTUL is a feasible outcome tool that adds to assessments such as SSPRO. Skin thickening is correlated with HRQoL, particularly in early disease.

中文翻译：

---

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 SSc 中上肢皮肤厚度患者自我评估 （PASTUL） 问卷，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 指定了对应于改良 Rodnan 皮肤评分 （mRSS） 的八个部位的皮肤分级。通过将 PASTUL 评分与 mRSS 进行比较来评估构建体效度。使用 EuroQoL 5 维度 5 水平 （EQ5D5L） 和 Leeds SSc QoL 问卷评估 HRQoL。此外，探讨了 PASTUL 与硬皮病皮肤患者报告结果 （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 （s.d. 13.9），80.6% 为女性 （n = 158），平均病程 11.9 年 （s.d. 9.9），110 名 （56.1%） 患有 lcSSc 和 81 名 （41.3%） 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 [类内相关系数 （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 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 （s.d. 3.7）。结论 PASTUL 是一种可行的结果工具，可添加到 SSPRO 等评估中。皮肤增厚与 HRQoL 相关，尤其是在疾病早期。