Abstract：
Sporadic inclusion body myositis (IBM) is a debilitating disease which leads to impaired ambulation and loss of hand function. Yale IBM Registry (IBMR) was launched in November 2016 to address the knowledge gap in IBM natural history data. The registry interface provides an IBM personalized index calculator (IBM-PIC) based on the IBM-functional rating scale (IBM-FRS). While the calculator is based on the IBM-FRS, it has not been directly compared to the IBM-FRS score. Therefore, in this study, we compared the patient-reported IBM-PIC score from this calculator with the physician-obtained IBM-FRS score.
IBM-FRS was administered over the phone within two weeks of their most recent IBM-PIC entry in the IBMR to 35 participants. To compare the agreement between IBM-FRS and IBM-PIC scores, Interrater Correlation Coefficient (ICC) analysis was performed. For individual questions, Fleiss Kappa statistics was used.
Thirty-five active IBM-PIC users participated. Eighty percent of the participants were men, and 91% were White Caucasians. The reported IBM-FRS score of this group was 23.5 ± 7.4 (range 1-38). The Interrater Correlation Coefficient (ICC) between the physician-administered IBM-FRS score and the IBM-PIC was 0.98 (0.96-0.99). There was moderate to substantial agreement on all the questions on IBM-FRS except for handwriting and fine motor skills.
IBM-PIC is a reliable indicator of the IBM-FRS score obtained by the physician. It is anticipated that this online platform will be a valuable tool for assessing IBM severity and monitoring disease progression remotely both in clinical practice and research studies.
IBM-FRS was administered over the phone within two weeks of their most recent IBM-PIC entry in the IBMR to 35 participants. To compare the agreement between IBM-FRS and IBM-PIC scores, Interrater Correlation Coefficient (ICC) analysis was performed. For individual questions, Fleiss Kappa statistics was used.
Thirty-five active IBM-PIC users participated. Eighty percent of the participants were men, and 91% were White Caucasians. The reported IBM-FRS score of this group was 23.5 ± 7.4 (range 1-38). The Interrater Correlation Coefficient (ICC) between the physician-administered IBM-FRS score and the IBM-PIC was 0.98 (0.96-0.99). There was moderate to substantial agreement on all the questions on IBM-FRS except for handwriting and fine motor skills.
IBM-PIC is a reliable indicator of the IBM-FRS score obtained by the physician. It is anticipated that this online platform will be a valuable tool for assessing IBM severity and monitoring disease progression remotely both in clinical practice and research studies.
摘要：
散发性包涵体肌炎（IBM）是一种使人衰弱的疾病，会导致步行障碍和手功能丧失。耶鲁IBM注册中心（IBMR）于2016年11月推出，旨在解决IBM自然历史数据中的知识差距。注册表界面提供了一个基于IBM功能评级量表(IBM-FRS)的IBM个性化索引计算器(IBM-PIC)。虽然计算器基于IBM-FRS，它没有直接与IBM-FRS评分进行比较。因此,在这项研究中，我们将该计算器中患者报告的IBM-PIC评分与医师获得的IBM-FRS评分进行了比较.
IBM-FRS在最近一次IBM-PIC进入IBMR的两周内通过电话给35名参与者。要比较IBM-FRS和IBM-PIC分数之间的协议，进行评分者间相关系数(ICC)分析。对于个别问题，使用了FleissKappa统计数据。
35名活跃的IBM-PIC用户参加了会议。80%的参与者是男性，91%是白人白种人。该组报告的IBM-FRS评分为23.5±7.4（范围1-38）。医师管理的IBM-FRS评分与IBM-PIC之间的评分者间相关系数(ICC)为0.98(0.96-0.99)。除了手写和精细运动技能外，在IBM-FRS的所有问题上都达成了中等到实质性的共识。
IBM-PIC是医师获得的IBM-FRS评分的可靠指标。预计该在线平台将是在临床实践和研究中评估IBM严重程度和远程监测疾病进展的有价值的工具。
IBM-FRS在最近一次IBM-PIC进入IBMR的两周内通过电话给35名参与者。要比较IBM-FRS和IBM-PIC分数之间的协议，进行评分者间相关系数(ICC)分析。对于个别问题，使用了FleissKappa统计数据。
35名活跃的IBM-PIC用户参加了会议。80%的参与者是男性，91%是白人白种人。该组报告的IBM-FRS评分为23.5±7.4（范围1-38）。医师管理的IBM-FRS评分与IBM-PIC之间的评分者间相关系数(ICC)为0.98(0.96-0.99)。除了手写和精细运动技能外，在IBM-FRS的所有问题上都达成了中等到实质性的共识。
IBM-PIC是医师获得的IBM-FRS评分的可靠指标。预计该在线平台将是在临床实践和研究中评估IBM严重程度和远程监测疾病进展的有价值的工具。
