Wasfi Yasmine S, Rose Cecile S, Murphy James R, Silveira Lori J, Grutters Jan C, Inoue Yoshikazu, Judson Marc A, Maier Lisa A
Pulmonary, Allergy, and Critcal Care Division, Department of Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA, USA.
Chest. 2006 May;129(5):1234-45. doi: 10.1378/chest.129.5.1234.
Sarcoidosis is a granulomatous disorder primarily affecting the lung, but with frequent extrapulmonary organ involvement. There are no comprehensive scoring systems for sarcoidosis disease severity. Our goal was to develop and validate an objective and comprehensive sarcoidosis disease severity scoring system.
Three sarcoidosis experts reviewed clinical data on 104 patients with biopsy-confirmed sarcoidosis. Each expert independently scored disease severity using a visual analog scale. Interrater agreement was assessed. Univariate analysis was performed, and those variables with p values < or = 0.25 were used in backward regression multivariable analysis. A model was obtained including variables with a p value of < or = 0.15 to predict severity scores. This model was subsequently validated using an independent panel of three additional international experts.
Granuloma clinic at National Jewish Medical and Research Center.
A total of 104 patients with biopsy-confirmed sarcoidosis.
None.
Pairwise assessment of interrater agreement yielded high degrees of correlation with Spearman correlation coefficients of 0.86 to 0.89 and an intraclass correlation coefficient of 0.87. Univariate analysis showed that smoking status, immunosuppressive therapy, percent predicted for diffusing capacity of the lung for carbon monoxide (Dlco), FEV1, FVC, and total lung capacity, FEV1/FVC ratio, disease duration, sites of organ involvement, and African-American race were associated with mean severity score. The multivariable model included cardiac and neurologic involvement, current therapy with noncorticosteroid immunosuppressive agents, Dlco percent predicted, FEV1/FVC ratio, African-American race, FVC percent predicted, and skin involvement. This model was validated using additional reviewer scores yielding Spearman correlation coefficients of 0.66 to 0.76 and an intraclass correlation coefficient of 0.74.
We derived an objective disease severity scoring system that incorporates data on demographics, pulmonary function, and organ involvement to produce a whole-body sarcoidosis assessment. This preliminary tool has potential applicability in the assessment of disease severity in sarcoidosis research.
结节病是一种主要影响肺部的肉芽肿性疾病，但常累及肺外器官。目前尚无针对结节病疾病严重程度的综合评分系统。我们的目标是开发并验证一种客观、全面的结节病疾病严重程度评分系统。
三位结节病专家回顾了104例经活检确诊的结节病患者的临床资料。每位专家使用视觉模拟量表独立对疾病严重程度进行评分。评估评分者间的一致性。进行单因素分析，将p值≤0.25的变量用于向后回归多变量分析。获得一个包含p值≤0.15的变量的模型以预测严重程度评分。随后，该模型由另外三位国际专家组成的独立小组进行验证。
国家犹太医学与研究中心的肉芽肿门诊。
总共104例经活检确诊的结节病患者。
无。
评分者间一致性的成对评估显示，Spearman相关系数为0.86至0.89，组内相关系数为0.87，具有高度相关性。单因素分析表明，吸烟状况、免疫抑制治疗、一氧化碳弥散能力（Dlco）预计值百分比、第一秒用力呼气容积（FEV1）、用力肺活量（FVC）、肺总量、FEV1/FVC比值、疾病持续时间、器官受累部位以及非裔美国人种族与平均严重程度评分相关。多变量模型包括心脏和神经受累、当前使用非皮质类固醇免疫抑制剂治疗、Dlco预计值百分比、FEV1/FVC比值、非裔美国人种族、FVC预计值百分比以及皮肤受累。使用其他评审者的评分对该模型进行验证，Spearman相关系数为0.66至0.76，组内相关系数为0.74。
我们得出了一种客观的疾病严重程度评分系统，该系统纳入了人口统计学、肺功能和器官受累的数据，以对全身结节病进行评估。这个初步工具在结节病研究中评估疾病严重程度方面具有潜在的适用性。
