O. Meyer
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引用次数: 45

摘要

系统性狼疮是一种自身免疫性疾病,以皮肤-关节、内脏(肾脏、神经系统、浆膜)和血液学病变为特征。由于观察到的各种症状,提出了分类标准;这些对于队列比较是有用的,但对于个体诊断是不够的。对主要临床表现进行分组可以区分良性形式(主要是皮肤-关节和浆液(胸膜心包炎)类型)和严重的内脏、肾脏、中枢神经系统、血液学(血小板减少症、溶血性贫血)和血栓形成形式。免疫学诊断是基于高水平的抗核抗体和较少见的天然抗dna抗体或抗核小体的证据。其他抗体对诊断也很有用,尽管它们很少被发现:抗sm抗体和抗核糖体抗体。在三分之一的病例中,抗磷脂抗体与动脉或静脉血栓并发症或产科事故有关。这种疾病通过连续发作自发发展,或多或少具有退行性。预后取决于适当治疗后内脏后遗症的严重程度,死亡率降低至疾病持续时间每年1%。治疗的基础是对症治疗(阿司匹林、降压药、降血脂药、抗凝血药)、长效药物(合成抗疟药、甲氨蝶呤等)、根据临床表现的严重程度给药的皮质激素和免疫抑制药物(环磷酰胺、硫唑嘌呤、霉酚酸酯)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Lupus érythémateux systémique

The systemic lupus is an autoimmune disease characterized by cutaneous-articular, visceral (kidney, nervous system, serous membranes), and haematological lesions. Due to the various groups of symptoms that have been observed, classification criteria have been proposed; these are useful for cohort comparisons, but are not adequate for individual diagnosis. Grouping main clinical manifestations allows to distinguish benign forms – mainly the cutaneous-articular, and the serous (pleuropericarditis) types – from the severe visceral, renal, central neurological, haematological (thrombopenia, haemolytic anaemia), and thrombotic forms. The immunological diagnosis is based on the evidence of high levels of antinuclear antibodies and, less frequently, of native anti-DNA antibodies, or antinucleosome. Other antibodies are useful for the diagnosis, despite their rareness: anti-Sm, and anti-ribosome antibodies. In one case out of three, antiphospholipid antibodies are associated with arterial or venous thrombotic complications, or with obstetrical accidents. The disease evolves spontaneously, by successive attacks, more or less regressive. Prognosis depends on the severity of the visceral sequelae after adequate treatment, with the mortality reduced to 1% per year of disease duration. Therapy is based on symptomatic treatments (aspirin, antihypertensive agents, hypolipidemic agents, anticoagulant drugs), long-acting medications (synthetic antimalarial drugs, methotrexate,…), corticoids dosed according to the severity of clinical manifestations, and immunosuppressive drugs (cyclophosphamide, azathioprin, mycophenolate).

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