Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being
Natural history of systemic sclerosis and the assessment of disease activity, severity, functional status, and psychologic well-being
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DOI：
10.1016/s0889-857x(03)00023-1
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发表时间：
2003-05-01
影响因子：
2.3
通讯作者：
Medsger, TA
中科院分区：
文献类型：
--
作者：
Medsger, TA
系统性硬化症（SSc）的自然史最好通过将患者分为弥漫性皮肤受累或有限皮肤受累的患者来理解;这些子集中的每一个都可以进一步分为早期和晚期疾病。这四个阶段中的每一个都具有特征性的临床和实验室特征。典型的炎症表现（如肌腱摩擦、腕管综合征）出现在早期疾病中，而代表血管和其他器官结构变化的表现（如关节挛缩、毛细血管扩张、钙质沉着）主要出现在晚期疾病中。测量SSc活动和损害的初步方法已经公布，但需要外部验证和完善。在评估功能状态的患者完成的工具中，健康评估问卷的残疾指数是使用最广泛的，具有表面和结构效度、可靠性和变化敏感性，是生存率的准确预测因子。
The natural history of systemic sclerosis (SSc) is best understood by dividing patients into those who have diffuse cutaneous involvement or limited cutaneous involvement; each of these subsets can be further divided into early and late disease. Each of these four stages has characteristic clinical and laboratory features. Findings that are typical of inflammation (eg, tendon friction rubs, carpal tunnel syndrome) appear in early disease, whereas those that represent vascular and other organ structural changes (eg, joint contractures, telangiectasias, calcinosis) occur predominantly in late disease. Preliminary methods to measure SSc activity and damage have been published but require external validation and refinement. Among patient-completed instruments that assess functional status, the disability index of the Health Assessment Questionnaire is the most widely used and has face and construct validity, reliability, and sensitivity to change and is an accurate predictor of survival.