Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder. Diagnosing AOSD can be challenging, as disease presentation and clinical course are highly heterogeneous. For unclear reasons, a few patients develop life-threatening complications. Our objective was to determine whether these cases resulted from therapeutic delay or could represent a peculiar AOSD subset. We conducted a multicentre retrospective study of 20 AOSD patients with organ failure requiring intensive care unit admission and 41 control AOSD patients without organ failure. Clinico-biological data at hospital admission were explored using supervised analyses and unsupervised dimension reduction analysis (factor analysis of mixed data, FAMD). Disease duration before admission was shorter in patients with life-threatening AOSD (median, 10 vs 20 days, p = 0.007). Disease duration before AOSD therapy initiation also tended to be shorter (median, 24 vs 32 days, p = 0.068). Despite this shorter disease duration, FAMD, hierarchical clustering and univariate analyses showed that these patients exhibited distinctive characteristics at first presentation, including younger age; higher frequency of splenomegaly, liver, cardiac and/or lung involvement; less frequent arthralgia; and higher ferritin level. In multivariate analysis, 3 parameters predicted life-threatening complications: lack of arthralgia, younger age and shorter time between fever onset and hospitalisation. This study suggests that life-threatening complications of AOSD occur very early, in a peculiar subset, which we propose to name catastrophic adult-onset Still’s disease (CAOSD). Its exact burden may be underestimated and remains to be clarified through large multicentre cohorts. Further studies are needed to identify red flags and define the optimal therapeutic strategy.

中文翻译：

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灾难性成人斯蒂尔病作为一个独特的威胁生命的临床亚群：带有降维分析的病例对照研究

成人斯蒂尔病 (AOSD) 是一种罕见的全身性炎症性疾病。诊断 AOSD 可能具有挑战性，因为疾病表现和临床过程具有高度异质性。由于不明原因，少数患者会出现危及生命的并发症。我们的目标是确定这些病例是由治疗延迟引起的，还是可能代表一个特殊的 AOSD 子集。我们对 20 名需要进入重症监护病房的器官衰竭 AOSD 患者和 41 名没有器官衰竭的对照 AOSD 患者进行了一项多中心回顾性研究。使用监督分析和无监督降维分析（混合数据的因子分析，FAMD）探索入院时的临床生物学数据。威胁生命的 AOSD 患者入院前的病程较短（中位数，10 天 vs 20 天，p = 0.007）。AOSD 治疗开始前的疾病持续时间也往往更短（中位数，24 天 vs 32 天，p = 0.068）。尽管疾病持续时间较短，但 FAMD、层次聚类和单变量分析表明，这些患者在首次就诊时表现出独特的特征，包括年龄较小；脾肿大、肝脏、心脏和/或肺部受累的频率更高；不太频繁的关节痛; 和更高的铁蛋白水平。在多变量分析中，3 个参数预测了危及生命的并发症：无关节痛、年龄较小以及发热和住院之间的时间较短。这项研究表明，威胁生命的 AOSD 并发症发生得很早，在一个特殊的子集中，我们建议将其命名为灾难性成人斯蒂尔病 (CAOSD)。它的确切负担可能被低估了，仍有待通过大型多中心队列来澄清。需要进一步的研究来识别危险信号并确定最佳治疗策略。