A disease severity scale for systemic sclerosis: development and testing.
A disease severity scale for systemic sclerosis: development and testing.
复制标题
系统性硬化症疾病严重程度量表：开发和测试。
作者：
T. Medsger;A. Silman;V. Steen;C. Black;A. Åkesson;P. Bacon;C. A. Harris;S. Jabłońska;M. Jayson;S. Jimenez;T. Krieg;Leroy Ec;P. Maddison;M. Russell;R. Schachter;F. Wollheim;H. Zacharaie
目的
开发和测试系统性硬化症（SSc，硬皮病）单个器官受累的严重程度量表。
方法
一个国际研究小组完成了以下任务：（1）编制了一份词汇表，其中包括9个可能受影响的器官系统的所有相关变量;（2）收集了前瞻性数据，以确定每个拟议变量的可行性和实用性;（3）修订了最初的变量清单;（4）确定各变量与死亡率的关系（发病率的代理）使用现有的综合纵向硬皮病数据库中的579名患者;（5）通过讨论和共识为每个器官系统制定了严重程度分级量表;（6）使用来自同一数据库的680名患者的独立组对该量表进行外部验证。
结果
9个器官特异性严重程度量表从0（没有记录的参与）到4（终末期疾病）。完成量表所需的数据对于所有医生来说都是相对容易和实用的。
结论
这个初步的严重程度量表将有助于在一个时间点和纵向评估个体患者的疾病严重程度状态。严重程度量表将有助于临床试验的设计和实施以及研究人群的相互比较。该量表将作为制定硬皮病疾病活动指数的框架。
OBJECTIVE
To develop and test a severity scale for individual organ involvements in systemic sclerosis (SSc, scleroderma).
METHODS
An international study group completed the following tasks: (1) developed a glossary of terms including all pertinent variables for 9 potentially affected organ systems; (2) collected prospective data to determine the feasibility and practicality of each proposed variable; (3) revised the initial list of variables; (4) determined the association of each variable with mortality (a proxy for morbidity) using 579 patients in an existing comprehensive longitudinal scleroderma databank; (5) developed a severity grading scale for each organ system by discussion and consensus; and (6) externally validated the scale using an independent group of 680 patients from the same databank.
RESULTS
Nine organ-specific severity scales were developed from 0 (no documented involvement) to 4 (endstage disease). The data required for scale completion are relatively easy and practical for all physicians to obtain.
CONCLUSION
This preliminary severity scale will be useful for assessing disease severity status in individual patients both at one point in time and longitudinally. The severity scale will assist in the design and conduct of clinical trials and the comparison of study populations with one another. The scale will serve as a framework for developing a scleroderma disease activity index.