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系统性红斑狼疮的初始特征对一组地中海地区患者临床病程及生存情况的影响

Contribution of the initial features of systemic lupus erythematosus to the clinical evolution and survival of a cohort of Mediterranean patients.

作者信息

Buján S, Ordi-Ros J, Paredes J, Mauri M, Matas L, Cortés J, Vilardell M

机构信息

Internal Medicine Service, Hospital General Vall d'Hebron, Barcelona, Spain.

出版信息

Ann Rheum Dis. 2003 Sep;62(9):859-65. doi: 10.1136/ard.62.9.859.

Abstract

BACKGROUND

Systemic lupus erythematosus has a wide spectrum of immunological and clinical manifestations. Its course is characterised by exacerbations which may result in mortality or morbidity to vital organs/systems.

OBJECTIVE

To determine clear and early prognostic markers to avoid further complications.

METHODS

245 adult patients diagnosed between January 1978 and March 2001 were studied. Clinical manifestations and laboratory findings both at onset and during the clinical course were collected. The number, type, and severity of the flares were also noted. Statistical analyses between disease features at onset, subsequent flares, and mortality were performed.

RESULTS

239 patients entered the study. Their mean age at onset was 30 years. The mean time between onset and diagnosis was 36 months and the mean evolution time was 114 months. 205 patients developed 915 flares; 205 (22.4%) of these flares were major flares, and affected 110 patients. Cardiac, neurological, or renal affection at onset were associated with a higher probability of developing cardiac (p=0.022), neurological (p<0.001), and renal (p<0.001) exacerbations, respectively, during the evolution. Lupus anticoagulant (LA) and anticardiolipin antibodies (aCL) were predictors of stroke (aCL, p=0.000; LA, p=0.001). Age at diagnosis (p=0.003) and valvular disease at onset (p=0.008) were independent predictors of low survival.

CONCLUSIONS

Renal, cardiac, or neurological involvement and the presence of LA or aCL positivity at onset were predictors of renal, cardiac, or neurological flares, respectively. Age and valvular involvement at onset were found to be independent adverse outcome predictors for low survival.

摘要

背景

系统性红斑狼疮具有广泛的免疫和临床表现。其病程的特点是病情加重,可能导致重要器官/系统的死亡或发病。

目的

确定明确且早期的预后标志物,以避免进一步的并发症。

方法

对1978年1月至2001年3月期间诊断的245例成年患者进行研究。收集发病时及临床病程中的临床表现和实验室检查结果。还记录了病情发作的次数、类型和严重程度。对发病时的疾病特征、随后的病情发作和死亡率进行了统计分析。

结果

239例患者进入研究。他们发病时的平均年龄为30岁。发病与诊断之间的平均时间为36个月,平均病程为114个月。205例患者出现了915次病情发作;其中205次(22.4%)为重度发作,累及110例患者。发病时心脏、神经或肾脏受累分别与病程中发生心脏(p=0.022)、神经(p<0.001)和肾脏(p<0.001)病情加重的可能性较高相关。狼疮抗凝物(LA)和抗心磷脂抗体(aCL)是中风的预测指标(aCL,p=0.000;LA,p=0.001)。诊断时的年龄(p=0.003)和发病时的瓣膜疾病(p=0.008)是低生存率的独立预测指标。

结论

发病时肾脏、心脏或神经受累以及LA或aCL阳性分别是肾脏、心脏或神经病情发作的预测指标。发现发病时的年龄和瓣膜受累是低生存率的独立不良预后预测指标。

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