Factors affecting survival in juvenile systemic sclerosis
Factors affecting survival in juvenile systemic sclerosis
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DOI：
10.1093/rheumatology/ken388
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发表时间：
2009-02-01
期刊：
影响因子：
5.5
通讯作者：
Zulian, F.
中科院分区：
文献类型：
--
作者：
Martini, G.;Vittadello, F.;Zulian, F.
目标.为了确定人口统计学、临床和免疫学特征是否可以预测青少年SSc（JSSc）的结局。我们分析了从全球儿科风湿病中心收集的JSSc患者的临床和实验室特征。首先进行单因素检验，确定与死亡结局显著相关的特征，然后进行多因素Logistic回归分析，确定死亡的预测因素。来自40个中心的134例患者符合分析条件。16例患者死亡，并迅速致命的过程中观察到的大多数：4/16死亡1年后诊断和10/16在5 yrs内。在诊断的那一刻，结果不佳的患者表现出显着较高的频率的内部器官的参与，特别是心脏，呼吸和胃肠道系统。皮肤、血管和肌肉骨骼受累的实体，自身抗体谱和实验室检查均无显著差异。多变量分析显示以下因素是死亡率的重要预测因素：胸部X线片显示的纤维化[比值比（OR）11.2]、肌酐水平升高（OR 22.7）和心包炎（OR 41.3），而诊断时疾病持续时间短则具有保护作用（OR 0.3）。所有具有JSSc和致死性结局的患者均受到弥漫性疾病的影响，其中大多数患者表现出非常快速的进展和内脏受累的早期体征。这表明，在儿童中，SSc可能有两种可能的过程：快速发展的内部器官衰竭导致严重残疾并最终死亡，或疾病的缓慢过程，死亡率较低。
Objectives. To determine whether demographic, clinical and immunological features may predict the outcome in juvenile SSc (JSSc).Methods. Clinical and laboratory characteristics of patients with JSSc collected from paediatric rheumatology centres worldwide were analysed. First, univariate tests identified those features significantly related with fatal outcome, and then multivariate logistic regression analysis was applied to determine the predictors of mortality.Results. One hundred and thirty-four patients from 40 centres were eligible for the analysis. Sixteen patients died and a rapidly fatal course was observed in most of them: 4/16 died within 1 yr after diagnosis and 10/16 within 5 yrs. At the moment of diagnosis, patients with poor outcome showed a significantly higher frequency of internal organ involvement, particularly cardiac, respiratory and gastrointestinal systems. No significant difference emerged for entity of skin, vascular and musculo-skeletal involvement, nor for auto-antibodies profile and laboratory tests. Multivariate analysis showed the following factors to be significant predictors of mortality: fibrosis on chest X-rays [odds ratio (OR) 11.2], raised creatinine levels (OR 22.7) and pericarditis (OR 41.3), while a short disease duration at diagnosis conferred protection (OR 0.3).Conclusions. All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality.