Singh S, Kumar L, Joshi K
Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Clin Rheumatol. 2002 Nov;21(6):462-5. doi: 10.1007/s100670200116.
Over the last 10 years we have seen 31 children with systemic lupus: 10 (32%) of these have died. The commonest primary determinant of mortality was uncontrolled disease activity (60%), possibly contributed to by late referrals leading to delays in diagnosis and the institution of therapy. Thromboembolic disease was responsible for two deaths and tuberculosis for one. The major contributory factors were nephritis in two patients and myocarditis, endocarditis, lupus pneumonia and CNS disease in one patient each. Concomitant infections, predominantly nosocomial, occurred in a significant proportion of patients (40%) but were only cofactors in mortality. Autopsies were done in three cases. These patterns of death are significantly different from those seen in the developed world, where disease activity has ceased to be an important factor owing to early recognition of cases. Avoiding late referrals and delays in diagnosis can reduce mortality in childhood-onset lupus.
在过去10年里,我们诊治了31例系统性红斑狼疮患儿:其中10例(32%)死亡。最常见的死亡首要决定因素是疾病活动未得到控制(60%),这可能是由于转诊延迟导致诊断和治疗延误所致。血栓栓塞性疾病导致2例死亡,结核病导致1例死亡。主要促成因素包括2例患者的肾炎以及各1例患者的心肌炎、心内膜炎、狼疮性肺炎和中枢神经系统疾病。相当一部分患者(40%)发生了合并感染,主要是医院感染,但感染只是死亡的辅助因素。对3例患者进行了尸检。这些死亡模式与发达国家所见的模式有显著不同,在发达国家,由于病例得到早期识别,疾病活动已不再是一个重要因素。避免转诊延迟和诊断延误可降低儿童期起病的狼疮的死亡率。