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希腊的系统性红斑狼疮。临床特征、病情发展及转归:对292例患者的描述性分析

Systemic lupus erythematosus in Greece. Clinical features, evolution and outcome: a descriptive analysis of 292 patients.

作者信息

Vlachoyiannopoulos P G, Karassa F B, Karakostas K X, Drosos A A, Moutsopoulos H M

机构信息

Department of Internal Medicine, School of Medicine, University of Ioannina, Greece.

出版信息

Lupus. 1993 Oct;2(5):303-12. doi: 10.1177/096120339300200505.

DOI:10.1177/096120339300200505
PMID:8305923
Abstract

The purpose of this study was the descriptive analysis of patients with systemic lupus erythematosus (SLE) with a particular focus on initial clinical features, evolution and outcome of disease, prevalence of clinical and serological manifestations and identification of clinicoserological associations indicative of renal and CNS involvement. The methodology applied was the following: retrospective analysis of the clinical charts of 292 unselected patients (246 female (84.2%) and 46 male (15.7%)) with SLE examined between 1982 and 1992. Multivariate analysis and hierarchical log linear models were used to examine for clinicoserological associations. Descriptive analysis was based on the prevalence of main clinicoserological features and disease outcome. The outcome was examined on the basis of the number of flares, the presence of chronic renal failure, the presence of central nervous system (CNS) involvement with subsequent disability and deaths. Flares were considered the severe alterations in disease status, requiring additional therapy to be controlled. The disease begins most frequently in the second and third decade of life with cutaneous and joint manifestations, while renal and CNS involvement developed later. The prevalence of serious renal, pulmonary and CNS involvement as well as the prevalence of RF, anti-Sm and anti-nRNP antibodies remain low. Multivariate analysis revealed the associations of renal involvement with leukopenia and serositis, of anti-Sm with leukopenia, of secondary Sjogren's syndrome with RF and of thromboembolic events with anticardiolipin antibodies. Patients with childhood onset SLE have a higher tendency for developing renal involvement than adult onset SLE patients. In addition, anti-Ro(SSA) antibodies were associated with anti-La(SSB) and RF, while anti-Sm antibodies were associated with anti-nRNP and RF. Flares occurred with a frequency of 0.07 per patient per year. Only 63.6% of flares were accompanied by positive anti-dsDNA reactivities. Reported deaths were 0.0047 per patient per year. Hierarchical log linear models indicated that the main variables of the disease were sufficient to describe our disease model and that the order of the interaction between the variables was insignificant. We conclude that the prevalence of various clinical features associated with SLE is similar, although the prevalence of CNS and pulmonary involvement as well as anti-Sm and anti-nRNP antibodies are less prominent in Greek SLE patients than that reported in the literature. The various clinicoserological associations detected do not appear to be of major significance as they are not powerful enough to subgroup the disease.

摘要

本研究的目的是对系统性红斑狼疮(SLE)患者进行描述性分析,特别关注疾病的初始临床特征、演变及转归、临床和血清学表现的患病率,以及确定提示肾脏和中枢神经系统受累的临床血清学关联。所采用的方法如下:对1982年至1992年间检查的292例未经选择的SLE患者(246例女性(84.2%)和46例男性(15.7%))的临床病历进行回顾性分析。采用多变量分析和分层对数线性模型来检测临床血清学关联。描述性分析基于主要临床血清学特征的患病率和疾病转归。根据病情复发次数、慢性肾衰竭的存在、中枢神经系统(CNS)受累及随后的残疾和死亡情况来评估转归。病情复发被视为疾病状态的严重改变,需要额外治疗来控制。疾病最常始于生命的第二个和第三个十年,表现为皮肤和关节症状,而肾脏和中枢神经系统受累则出现较晚。严重肾脏、肺部和中枢神经系统受累的患病率以及类风湿因子(RF)、抗Sm和抗nRNP抗体的患病率仍然较低。多变量分析显示,肾脏受累与白细胞减少和浆膜炎相关,抗Sm与白细胞减少相关,继发性干燥综合征与RF相关,血栓栓塞事件与抗心磷脂抗体相关。儿童期发病的SLE患者比成人期发病的SLE患者发生肾脏受累的倾向更高。此外,抗Ro(SSA)抗体与抗La(SSB)和RF相关,而抗Sm抗体与抗nRNP和RF相关。每位患者每年病情复发的频率为0.07。只有63.6%的病情复发伴有抗双链DNA反应阳性。报告的死亡率为每位患者每年0.0047。分层对数线性模型表明,该疾病的主要变量足以描述我们的疾病模型,且变量之间相互作用的顺序无关紧要。我们得出结论,与SLE相关的各种临床特征的患病率相似,尽管希腊SLE患者中枢神经系统和肺部受累以及抗Sm和抗nRNP抗体的患病率比文献报道的要低。检测到的各种临床血清学关联似乎没有重大意义,因为它们不足以对疾病进行亚组分类。

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