系统性红斑狼疮的心脏病变

系统性红斑狼疮是一种病因未明的自身免疫介导的炎症性结缔组织病，心脏是其累及的重要器官之一。心脏病变现已成为影响患者生存率以及病死率的重要因素。本文综述了系统性红斑狼疮患者主要的心脏病变。     

自身免疫病的生物学治疗进展

自身免疫病是自身免疫系统功能紊乱产生相应抗体，导致靶器官或多系统损伤，临床表现不一的一组疾病。由于其治愈率低、致残和致死率高，已日益受到重视。关于自身免疫病的治疗，目前尚无十分令人满意的方法，近年来兴起的生物学治疗带来了新的希望。为此，主要论述了自身免疫病尤其是系统性红斑狼疮和类风湿关节炎的生物学治疗进展。     

系统性红斑狼疮心脏损伤特点概述

系统性红斑狼疮是累及多脏器的自身免疫介导的炎症性结缔组织疾病。心脏受累率高,是影响SLE患者的生存质量和远期预后的重要因素。本文对SLE心脏损伤主要表现予以概述。     

系统性红斑狼疮心脏损伤特点概述1王文芳,邓丹琪

系统性红斑狼疮是累及多脏器的自身免疫介导的炎症性结缔组织疾病。心脏受累率高,是影响SLE患者的生存质量和远期预后的重要因素。本文对SLE心脏损伤主要表现予以概述。     

有关系统性红斑狼疮诊疗中的若干问题

系统性红斑狼疮（SLE）是一种极为复杂的自身免疫系性疾病，近年来诊疗水平有较大提高，在发达国家，其5年存活率已提高到95％，但因病因仍未阐明，故只能控制病情而不能根治。现将当前在诊治中的一些主要问题作如下介绍。     

系统性红斑狼疮与甲状腺疾病

系统性红斑狼疮是一种异质性强的自身免疫性疾病，血清中产生以抗核抗体为代表的多种自身抗体，可引起全身器官及系统损害，患者死亡的原因主要为肾衰竭、狼疮脑病和严重感染。伴发甲状腺疾病虽然不是患者死亡的主要原因，但其发病率越来越高，研究系统性红斑狼疮与甲状腺疾病的相关性有助于加深对系统性红斑狼疮疾病特征的认识，提高患者预后。该文就系统性红斑狼疮与甲状腺疾病作一综述。     

自身免疫病的生物学治疗进展

自身免疫病是自身免疫系统功能紊乱产生相应抗体,导致靶器官或多系统损伤,临床表现不一的一组疾病。由于其治愈率低、致残和致死率高,已日益受到重视。关于自身免疫病的治疗,目前尚无十分令人满意的方法,近年来兴起的生物学治疗带来了新的希望。为此,主要论述了自身免疫病尤其是系统性红斑狼疮和类风湿关节炎的生物学治疗进展。     

DNA酶Ⅰ缺陷与SLE相关性的研究进展

系统性红斑狼疮的病因复杂，发病机理尚未完全阐明，研究表明DNA核蛋白在系统性红斑狼疮发病中起重要作用。而DNA酶I是其谢的主要核酸酶，综述了关于DNA酶I缺陷在系统性红斑狼疮发病中可能的致病机理，为进一步研究系统性红斑狼疮的病因提供一个思路。     

SLE患者肝损害与相关症状及生物学指征的关系

系统性红斑狼疮（systemic lupus erythematosus，sLE）是一种病因未明，自身免疫介导的累及多系统多器官，临床表现复杂炎症性结缔组织病。严重威胁患者的健康甚至生命，对其所引起损害发生率的研究，可以对该病的诊治过程进行有效监测和预防提供帮助。     

红斑狼疮的诊断和治疗

红斑狼疮(LE)是自身免疫介导的、以炎症为表现的结缔组织疾病，常累及多个脏器和器官，临床表现较为复杂而多变．常以缓解和复发交替出现，病程呈慢性迁延．多见于女性。有时与其他结缔组织病如皮肌炎、硬皮病等重叠出现。LE是一个病谱疾病，病谱的一端为DLE，病变主要限于皮肤，另一端为SLE伴弥漫增殖性狼疮性肾炎。中间有很多亚型，如DDLE、LET、深在性红斑狼疮、SCLE、新生儿红斑狼疮、ANA阴性的SLE等。     

系统性红斑狼疮脱发的研究进展

脱发是SLE常见的临床表现之一。SLE脱发可表现为多种类型,如狼疮发、非瘢痕性斑状脱发、弥漫性休止期脱发、盘状红斑狼疮型脱发等,不同类型的脱发在临床表现和组织病理学方面有其各自的特点。SLE脱发与疾病活动性有一定的相关性。目前SLE脱发的发病机制尚未明确,自身免疫性炎症和血管炎造成的局部微环境的改变、毛发营养不良和毛囊周期失调均有可能参与其中。     

病毒感染与系统性红斑狼疮相关性研究进展

系统性红斑狼疮(systemic lupus erythematosus,SLE)是自身免疫性疾病的原型,发病机制十分复杂,至今仍不明确。近年研究表明,病毒感染可能是该病的一个重要的病因,病毒感染不仅参与SLE的发生发展,还与SLE的免疫学、临床表现有相关性。随着病毒感染与SLE相关性的研究深入,SLE的治疗也从中获得了一些启示。     

生物制剂治疗系统性红斑狼疮新进展

系统性红斑狼疮是一种表现多样的慢性自身免疫病，可累及全身。糖皮质激素及免疫抑制剂为主的传统药物对部分SLE患者的疗效欠佳，且长期应用毒副作用大。生物制剂的出现为SLE治疗带来新的选择，临床应用中也取得了一定成效。本文就生物制剂在红斑狼疮临床治疗中的新进展作一综述。     

2019年系统性红斑狼疮研究新进展

【摘要】 系统性红斑狼疮是一种主要累及中青年女性的自身免疫性疾病，临床表现复杂多样，可累及多器官系统。系统性红斑狼疮病因、诊断与治疗的研究一直是该领域的热点。2019年，国内外研究者在其发病机制、诊断和评估以及治疗方面取得了一系列新进展，本文就主要代表性成果进行综述。     

系统性红斑狼疮男性患者临床特征研究进展

系统性红斑狼疮（SLE）是常见的自身免疫性疾病,女性SLE患病率明显高于男性,其临床特征存在性别差异。SLE男性患者临床表现谱与女性患者相似,但两者在受累器官、疾病严重程度、并发症及预后等方面存在差异,尤其SLE男性患者疾病严重程度和病死率明显高于女性患者。SLE男性患者治疗原则与女性相似,但在药物选择、累计剂量方面存在差异。本文对系统性红斑狼疮男性患者临床特征进行综述,以提高对男性SLE的早期识别与诊断能力。     

Development of the CLASI as an outcome instrument for cutaneous lupus erythematosus

Skin involvement is a frequent presenting manifestation of systemic lupus erythematosus (SLE). Cutaneous lupus erythematosus (CLE), frequently occurring without SLE, may be even more common than SLE. Until recently, clinical instruments to measure skin involvement in CLE did not exist, hampering clinical research in this field. In this paper the present authors describe outcome instruments for SLE and outline the considerations underlying the design and validation of an outcome instrument for CLE, the cutaneous lupus disease area and severity index. These studies serve as a model for development and validation of standardized instruments that can be applied to other cutaneous diseases, particularly autoimmune diseases, in order to facilitate epidemiologic studies and clinical trials.     

系统性红斑狼疮的皮肤外临床表现

系统性红斑狼疮（SLE）是一种复杂的自身免疫性疾病,以自身抗体形成免疫复合物为特点,可伴有全身多器官炎症和广泛临床表型,其中皮肤科的症状表浅、特异,较易为医务工作者所掌握,但其他系统的临床表现错综复杂,极易误诊和漏诊。该文主要归纳总结了SLE在皮肤外各个系统的临床特征,包括黏膜、关节、心血管、肺、血液、消化道、肝脏、肾脏、浆膜等,旨在加深对此病的认识,提高SLE的诊治水平。     

三氧化二砷在系统性红斑狼疮治疗中的应用

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种由遗传、环境、免疫等多种因素参与引起的多器官、多系统损害的自身免疫性疾病,其发病机制复杂,临床表现多样,病情多呈进行性发展。对于SLE的治疗,传统上主要以糖皮质激素联合免疫抑制剂为主,严重者辅以细胞毒药物或生物调节剂,但总体疗效欠佳,且不良反应较大。近年来,大量的基础研究和临床试验表明,免疫因素在SLE发病机制中起主要作用,三氧化二砷(ATO)能有效缓解、控制SLE的症状,并且对ATO治疗SLE的作用机制也进行了许多深入的研究。该文拟从ATO的药效特性、临床应用、作用机制等方面对ATO治疗SLE的研究现况做一概述。     

Elevated serum BAFF levels in patients with localized scleroderma in contrast to other organ-specific autoimmune diseases

Serum levels of B-cell activating factor belonging to the tumor necrosis factor family (BAFF), a potent B-cell survival factor, are elevated in patients with systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis (SSc). The objective of this study was to determine serum BAFF levels and relate the results to the clinical features in patients with organ-specific autoimmune diseases of the skin, such as localized scleroderma and autoimmune bullous diseases. Serum BAFF levels were examined by enzyme-linked immunosorbent assay in 44 patients with localized scleroderma, 20 with pemphigus vulgaris/pemphigus foliaceus, 20 with bullous pemphigoid and 30 healthy controls. Twenty patients with SSc and 20 with SLE were also examined as disease controls. Serum BAFF levels were elevated in localized scleroderma patients compared with healthy controls. Concerning localized scleroderma subgroups, patients with generalized morphea, the severest form of localized scleroderma, had higher serum BAFF levels than linear scleroderma or morphea patients. The BAFF levels of generalized morphea were comparable with those of SSc or SLE. Furthermore, serum BAFF levels correlated positively with antihistone antibody levels and the severity of skin lesion as well as the number of skin lesions. By contrast, serum BAFF levels were not significantly elevated in patients with pemphigus or pemphigoid. These results suggest that BAFF may be contributing to autoimmunity and disease development in localized scleroderma.     

Up-regulation of cellular FLICE-inhibitory protein in peripheral blood B lymphocytes in patients with systemic lupus erythematosus is associated with clinical characteristics

Background  Systemic lupus erythematosus (SLE) is an autoimmune disease which is involved in T- and B-lymphocyte–mediated autoimmunity. Apoptosis contributes to the maintenance of lymphocytes homeostasis and the deletion of autoreactive cells in SLE. Although there is evidence that cellular FLICE-inhibitory protein (c-FLIP), an antiapoptosis protein, is increased in human lupus T cells to keep them from apoptosis, but the expression of apoptosis-regulatory protein c-FLIP in SLE B lymphocytes remains unknown.
Aims  To study the expression of c-FLIP in peripheral blood B lymphocytes in SLE patients and to investigate the relationship among the expression of c-FLIP in peripheral blood B lymphocytes in SLE patients, clinical manifestation and the levels of interleukin-4 (IL-4) and IL-10.
Methods  In this study, we detected the expression of c-FLIP in peripheral blood B lymphocytes in SLE patients by flow cytometry and the levels of IL-4 and IL-10 in SLE serum samples by enzyme-linked immunosorbent assay and analysed their relationship with clinical characteristics.
Results  We observed a significantly higher percentage of c-FLIP in peripheral B cells in SLE patients with active disease when compared to inactive ones and healthy controls. And the expression of c-FLIP in lupus peripheral B cells showed positive correlations with SLEDAI, erythrocyte sedimentation rate, C-reactive protein, antinucleosome antibody titre, IL-4, and IL-10, and negative correlation with white blood cell count. Patients with lupus nephritis had higher levels of c-FLIP in peripheral B cells than patients without lupus nephritis.
Conclusion  Our data show that overexpression of c-FLIP is relevant to the activity and severity of SLE. Its overexpression might play a role in preventing B cell from apoptosis in SLE. The cause of c-FLIP overexpression may be due to the increase of IL-4 and IL-10 levels in SLE patients.