Pemphigus Disease Activity Measurements Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, and Pemphigus Vulgaris Activity Score
Pemphigus Disease Activity Measurements Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, and Pemphigus Vulgaris Activity Score
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DOI：
10.1001/jamadermatol.2013.8175
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发表时间：
2014-03-01
期刊：
影响因子：
10.9
通讯作者：
Chams-Davatchi, Cheyda
中科院分区：
文献类型：
--
作者：
Rahbar, Ziba;Daneshpazhooh, Maryam;Chams-Davatchi, Cheyda
重要性最近，天疱疮疾病面积指数（PDAI）、自身免疫性大疱性皮肤病强度评分（ABSIS）和寻常天疱疮活动评分（PVAS）的临床天疱疮疾病活动指数经验证与医生总体评估相关。已知抗桥粒芯糖蛋白（抗Dsg）自身抗体主要与天疱疮疾病活动相关。这些指标与抗-Dsg 1和抗-Dsg 3酶联免疫吸附测定值之间的相关性以前没有被评估过。目的评估大量寻常型天疱疮患者的PDAI、ABSIS和PVAS，并比较这些指标的评分者间信度和根据抗-Dsg值的收敛效度。和参与者一项横断面研究于2012年在一所大学的自身免疫性大疱性疾病转诊中心进行。研究了100例确诊寻常型天疱疮和临床天疱疮病变的患者（平均[SD]年龄，43.3 [1.7]岁;年龄范围，14-77岁;男女比例，1：3）。三位熟悉免疫大疱性疾病的皮肤科医生对患者进行评估。干预所有100例患者均采用PDAI、ABSIS和PVAS进行评估。三位皮肤科医生在同一天对每位患者的所有3项指标进行独立评分。主要结果和指标：分析评价者间的信度、抗Dsg滴度的一致性效度、病变类型和分布与疾病活动性的相关性、高滴度抗体的预测因子（多元回归分析），以及抗Dsg的2个滴度的测量的截止值，具有最佳曲线下面积，灵敏度，结果评价者间信度以PDAI最高，其次为ABSIS和PVAS（组内相关系数分别为0.98 [95%CI，0.97-0.98]、0.97 [95%CI，0.96-0.98]和0.93 [95%CI，0.90-0.95]）。PDAI的收敛效度最高，其次是PVAS和ABSIS（斯皮尔曼p = 0.67、0.52和0.33）。头、颈和躯干受累是抗Dsg 1抗体滴度升高的预测因子。结论和相关性在3个研究指标中，PDAI的有效性最高，推荐用于寻常型天疱疮等罕见病的多中心研究。
IMPORTANCE Recently, the clinical pemphigus disease activity indexes of Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS) were validated to correlate with physician global assessment. The antidesmoglein (anti-Dsg) autoantibodies are known to correlate mostly with pemphigus disease activity. The correlation between these indexes and anti-Dsg1 and anti-Dsg3 enzyme-linked immunosorbent assay values has not been previously evaluated.OBJECTIVES To evaluate the PDAI, ABSIS, and PVAS in a large number of patients with pemphigus vulgaris and to compare the interrater reliability of these indexes and the convergent validity according to anti-Dsg values.DESIGN, SETTING, AND PARTICIPANTS A cross-sectional study was performed in 2012 in a referral university center for autoimmune bullous diseases. One hundred patients with confirmed diagnoses of pemphigus vulgaris and clinical pemphigus lesions (mean [SD] age, 43.3 [1.7] years; age range, 14-77 years; female-male ratio, 1: 3) were studied. Three dermatologists familiar with immunobullous diseases and the indexes rated the patients.INTERVENTIONS All 100 patients were evaluated with the PDAI, ABSIS, and PVAS. Three dermatologists independently rated all 3 indexes for each of the patients on the same day. Serum anti-Dsg1 and anti-Dsg3 enzyme-linked immunosorbent assay values were measured simultaneously.MAIN OUTCOMES AND MEASURES Analyses of interrater reliabilities, convergent validities according to anti-Dsg titers, correlation between the distribution and types of lesions with disease activity, predictors of higher titers of antibody (multiple regression analysis), and cutoff values of measures for 2 titers of anti-Dsg with optimal area under the curve, sensitivity, and specificity were performed.RESULTS The interrater reliabilities were highest for the PDAI, followed by the ABSIS and the PVAS (intraclass correlation coefficients of 0.98 [95% CI, 0.97-0.98], 0.97 [95% CI, 0.96-0.98], and 0.93 [95% CI, 0.90-0.95], respectively). The convergent validity was highest for the PDAI, followed by the PVAS and the ABSIS (Spearman p = 0.67, 0.52, and 0.33, respectively). Head, neck, and trunk involvement were predictors of higher titers of anti-Dsg1.CONCLUSIONS AND RELEVANCE Among the 3 studied indexes, the PDAI had the highest validity and is recommended for use in multicenter studies for rare diseases, such as pemphigus vulgaris.