Ying Shuni, Lv Duo, Zhu Dingxian, Li Sheng, Ding Yuwei, Sun Chuanyin, Shi Yu, Fang Hong, Qiao Jianjun
Department of Dermatology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Department of Clinical Pharmacy, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
J Transl Autoimmun. 2022 Dec 22;6:100184. doi: 10.1016/j.jtauto.2022.100184. eCollection 2023.
To develop and validate a diagnostic score to identify adult-onset Still's disease (AOSD) in fever of unknown origin (FUO).
A single center, retrospective case-control study of inpatients with FUO from January 2018 to December 2021. Using clinical and laboratory data from 178 cases with AOSD and 486 cases with FUO, we developed an AOSD/FUO (AF) score with a Bayesian Model Averaging approach. AF score and Yamaguchi's criteria were evaluated by sensitivity, specificity, accuracy, and positive/negative predictive value for diagnosis of AOSD in developmental and validation samples.
Persistent pruritic eruptions (PPEs) in patients with rashes was higher in AOSD group than FUO group (52.3% vs 7.4%; < 0.01). PPEs yielded a specificity of 97.5% and a sensitivity of 44.9%. AF score = PPEs × 3.795+Evanescent rash × 2.774+Serum ferritin × 1.678+Myalgia × 0.958+Neutrophil count × 0.185+Platelet count × 0.004. A cut-off value ≥ 5.245 revealed the maximizing sensitivity of 88.7% and specificity of 95.8% in discriminating AOSD from FUO in the validation group. And AF score improved the accuracy from 82.6% to 93.3% compared with Yamaguchi's criteria.
We developed and validated a new score which can identify AOSD in FUO with higher classification accuracy than Yamaguchi's criteria. Future multi-centric prospective studies need to be designed to confirm the diagnosis value of AF score.
开发并验证一种诊断评分系统，以在不明原因发热（FUO）中识别成人斯蒂尔病（AOSD）。
对2018年1月至2021年12月期间因FUO住院的患者进行单中心回顾性病例对照研究。利用178例AOSD患者和486例FUO患者的临床和实验室数据，我们采用贝叶斯模型平均法开发了一种AOSD/FUO（AF）评分。在开发和验证样本中，通过敏感性、特异性、准确性以及诊断AOSD的阳性/阴性预测值对AF评分和山口标准进行评估。
有皮疹的患者中，AOSD组持续性瘙痒性皮疹（PPEs）的发生率高于FUO组（52.3%对7.4%；P<0.01）。PPEs的特异性为97.5%，敏感性为44.9%。AF评分=PPEs×3.795+一过性皮疹×2.774+血清铁蛋白×1.678+肌痛×0.958+中性粒细胞计数×0.185+血小板计数×0.004。在验证组中，截断值≥5.245时，区分AOSD与FUO的敏感性最高可达88.7%，特异性为95.8%。与山口标准相比，AF评分将准确性从82.6%提高到了93.3%。
我们开发并验证了一种新的评分系统，该系统在FUO中识别AOSD的分类准确性高于山口标准。未来需要设计多中心前瞻性研究来证实AF评分的诊断价值。
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