全身性红斑狼疮

title: 分类.
content: SLE临床表现复杂多样,可分为以下几种亚型:  多数呈隐匿起病,开始仅累及1~2个系统,表现为轻度的关节炎、皮疹、隐匿性肾炎、血小板减少性紫癜等,部分病人长期稳定在亚临床状态或轻型狼疮,部分病人可由轻型突然变为重症狼疮,更多的则由轻型逐渐出现多系统损害;也有一些病人一起病就累及多个系统,甚至表现为狼疮危象。
title: 发病机制.
content: SLE是一种异质性的疾病,真正的致病机制仍然未知,其根源是多因素的,由基因,自身抗体,免疫复合体,内分泌荷尔蒙,环境因子以及其他一些未知的因子等复杂的交亘反应所造成的。这些因子合作产生显著T协助细胞和B细胞的活化,引起一些多专一性(polyspecific)抗体的分泌。  「红斑性狼疮」可能是「抗SS-A/Ro抗体」所导致,但也可能是「抗Sm抗体」、「抗SS-B/ La抗体」等等所导致。目前相信这种疾病是由于案例本身产生自体免疫抗体造成特殊的细胞毒性伤害的一种自体免疫疾病,包括有溶血性贫血,血小板缺少症。有些会形成免疫复合体而形成免疫复合体发炎反应,包括有肾炎,皮肤炎,及中枢神经的发炎反应。  在这复杂的过程中,每一个因子都是必须的,但每一个分子单独都不足以引起此病的临床表现,不同因子间的相对重要性则因人而异。阳光里的紫外线能导致圆盘状狼疮的突然发作,甚至也可能引起首度的爆发。疲劳、生产、感染、某些药物(通常在中断用药后即复原)、紧张与压力、病毒感染、化学物品等等,也可能触发狼疮。病人中只有约百分之三十的病人因为对光敏感,必须避免晒太阳,其它大多数病人无须逃避阳光而影响日常社交生活。有些狼疮会发生在关节处,发病时又肿又痛。情况严重时,将影响脑、肾、心等器官的功能。当涉及中枢神经系统时,猝发症、健忘、精神病、抑郁都可能发生。  诊断口诀:面颊蝴蝶盘状斑,日光过敏关节炎。心包炎症狼疮肺,口腔溃疡肾病变。血液系统四减少,精神症状非少见。免疫标志细测定,抗核抗体最敏感。
title: 诊断标准.
content: 根据1972及1982年美国风湿医学会(American Rheumatism Association)标准,凡具有下列十一项准则中的任四项以上,可诊断为红斑性狼疮。
title: 治疗方法.
content: 美国食品和药物管理局(Food and Drug Administration, 简称FDA)已经批准了Human Genome Sciences Inc. 和葛兰素史克(Glaxosmithkline plc, GSK)的红斑狼疮新药Benlysta上市,这是50多年来首次开发出红斑狼疮治疗新药。

Summary: 全身性红斑狼疮(英语:systemic lupus erythematosus,简称英语:SLE)又称系统性红斑狼疮,是一种慢性的自体免疫性疾病。乃身体因不知明原因,促使免疫系统产生自身抗体攻击自身细胞和组织,导致发炎和组织损害。症状的严重程度因人而异,常见症状包含关节炎、发烧、胸痛、脱发、口腔溃疡、淋巴腺肿大、疲倦,及脸部红疹等等,本疾病的病情会随病程有所变化,病情较严重的时期称为「发作」(Flares),病情较轻微的时期则称「缓解」。 造成本疾病的原因尚不明朗,目前认为可能与激素、环境因子,及遗传有关。如果同卵双胞胎其中一个患病,另一个有24%的机会也会患病。其他可能的潜在危险因子还包含雌性激素、阳光、抽烟、维生素D缺乏症,以及其他感染因素。造成疾病的抗体一般为抗核抗体,并造成发炎。部分患者不易诊断,需结合患者症状辅以实验室检验才能确诊。除了本疾病以外,另还有很多其他类型的红斑性狼疮,包含圆盘状红斑狼疮、新生儿红斑狼疮,以及亚急性皮肤红斑狼疮。 迄今仍无根治方法,目前使用的治疗药物包含NSAID、皮质类固醇、免疫抑制剂、羟氯喹,以及氨甲蝶呤。替代疗法的结果并没有实质帮助。SLE的患者预期寿命比一般人短,并提升了心血管疾病的风险,为患者最常见的死因。现代的疗法可使80%的患者存活15年以上。罹患此病的孕妇具有较高的风险,但通常能够成功生产。 本疾病的发生率约为每10万人有20至70例,其中适婚年龄的女性的病例数为男性的九倍。罹病的年龄约于 15 至 45 岁之间。非裔、加勒比海人,及华裔的罹病率较高加索人为高。至于开发中国家的发生率目前仍未知。「红斑性狼疮」译自拉丁文「Lupus erythematosus」,其中「Lupus」一词指的是狼的意思,乃因18世纪期间人们相信该疾病是因狼咬而导致。

Lupus: Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary between people and may be mild to severe. Common symptoms include painful and swollen joints, fever, chest pain, hair loss, mouth ulcers, swollen lymph nodes, feeling tired, and a red rash which is most commonly on the face. Often there are periods of illness, called flares, and periods of remission during which there are few symptoms. The cause of SLE is not clear. It is thought to involve genetics together with environmental factors. Among identical twins, if one is affected there is a 24% chance the other one will be as well. Female sex hormones, sunlight, smoking, vitamin D deficiency, and certain infections are also believed to increase the risk. The mechanism involves an immune response by autoantibodies against a person's own tissues. These are most commonly anti-nuclear antibodies and they result in inflammation. Diagnosis can be difficult and is based on a combination of symptoms and laboratory tests. There are a number of other kinds of lupus erythematosus including discoid lupus erythematosus, neonatal lupus, and subacute cutaneous lupus erythematosus. There is no cure for SLE. Treatments may include NSAIDs, corticosteroids, immunosuppressants, hydroxychloroquine, and methotrexate. Although corticosteroids are rapidly effective, long term use results in side effects. Alternative medicine has not been shown to affect the disease. Life expectancy is lower among people with SLE. SLE significantly increases the risk of cardiovascular disease with this being the most common cause of death. With modern treatment about 80% of those affected survive more than 15 years. Women with lupus have pregnancies that are higher risk but are mostly successful. Rate of SLE varies between countries from 20 to 70 per 100,000. Women of childbearing age are affected about nine times more often than men. While it most commonly begins between the ages of 15 and 45, a wide range of ages can be affected. Those of African, Caribbean, and Chinese descent are at higher risk than white people. Rates of disease in the developing world are unclear. Lupus is Latin for "wolf": the disease was so-named in the 13th century as the rash was thought to appear like a wolf's bite.