Ziemek Jessica, Man Ada, Hinchcliff Monique, Varga John, Simms Robert W, Lafyatis Robert
Department of Medicine, Rheumatology Section, Boston University School of Medicine, Boston, MA and.
Department of Medicine, Rheumatology Section, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA.
Rheumatology (Oxford). 2016 May;55(5):911-7. doi: 10.1093/rheumatology/kew003. Epub 2016 Feb 15.
To determine how well skin symptoms considered specific to SSc are captured by patient reported outcomes currently used for assessing patients with SSc, the SHAQ, or skin disease, the Skindex-29; and how well these symptoms correlate with the extent of skin disease on physical exam and skin pathology.
SSc patients completed the scleroderma modification of the Health Assessment Questionnaire (SHAQ), Skindex-29 and a Skin Symptom Assessment questionnaire developed for this study. Correlations were assessed between the Skin Symptom Assessment and SHAQ, Skindex-29, modified Rodnan skin score, and skin pathological features including myofibroblast staining completed on the same date.
Tight, hard and rigid/stiff skin symptoms correlated moderately highly with the modified Rodnan skin score (r = 0.445, P = 0.0008; r = 0.486, P = 0.0002; and r = 0.488, P = 0.0002, respectively). Tight skin symptoms correlated moderately with myofibroblast infiltration (r = 0.544, P = 0.0023) and hyalinized collagen (r = 0.442, P = 0.0164), while both hard and rigid/stiff skin correlated moderately with inflammation (r = 0.401, P = 0.0310 and r = 0.513, P = 0.0045), myofibroblast infiltration(r = 0.480, P = 0.0084 and r = 0.527, P = 0.0033) and hyalinized collagen (r = 0.453, P = 0.0137 and r = 0.478, P = 0.0087), while the SHAQ was not found to correlate with any of these pathological changes. In contrast, painful skin symptoms correlated moderately with the SHAQ (r = 0.413, P = 0.0073), and with the three domains of Skindex-29: Symptoms, Emotions and Functioning. Skindex-29 indicates that dcSSc patient skin symptoms are nearly as severe as those of patients with psoriasis or atopic dermatitis.
Patient reported skin symptoms correlate with clinical and pathological measures in the skin. A validated patient reported skin symptom instrument might considerably improve evaluation of SSc skin disease.
确定目前用于评估系统性硬化症（SSc）患者的患者报告结局指标（SHAQ）或用于评估皮肤病的Skindex-29，对被认为是SSc特异性皮肤症状的捕捉程度；以及这些症状与体格检查中的皮肤疾病程度和皮肤病理学之间的关联程度。
SSc患者完成了健康评估问卷（HAQ）的硬皮病改良版（SHAQ）、Skindex-29以及为本研究开发的皮肤症状评估问卷。评估了皮肤症状评估与SHAQ、Skindex-29、改良的Rodnan皮肤评分以及同一天完成的包括肌成纤维细胞染色在内的皮肤病理特征之间的相关性。
紧绷、坚硬和僵硬的皮肤症状与改良的Rodnan皮肤评分中度高度相关（分别为r = 0.445，P = 0.0008；r = 0.486，P = 0.0002；r = 0.488，P = 0.0002）。紧绷的皮肤症状与肌成纤维细胞浸润（r = 0.544，P = 0.0023）和透明化胶原（r = 0.442，P = 0.0164）中度相关，而坚硬和僵硬的皮肤均与炎症（r = 0.401，P = 0.0310和r = 0.513，P = 0.0045）、肌成纤维细胞浸润（r = 0.480，P = 0.0084和r = 0.527，P = 0.0033）和透明化胶原（r = 0.453，P = 0.0137和r = 0.478，P = 0.0087）中度相关，而未发现SHAQ与这些病理变化中的任何一项相关。相比之下，疼痛的皮肤症状与SHAQ中度相关（r = 0.413，P = 0.0073），并与Skindex-29的三个领域：症状、情绪和功能相关。Skindex-29表明，弥漫性皮肤型系统性硬化症（dcSSc）患者的皮肤症状几乎与银屑病或特应性皮炎患者的症状一样严重。
患者报告的皮肤症状与皮肤的临床和病理指标相关。经过验证的患者报告皮肤症状工具可能会显著改善对SSc皮肤疾病的评估。
