伴有中枢神经系统损害的系统性红斑狼疮患者的脑电图分析

系统性红斑狼疮（SLE）是一种表现为多系统损害症状的慢性系统性自身免疫病。多见于15～40岁的女性。其临床表现可累及全身任何脏器，有报道显示，早期有25％的患者可引起中枢神经系统损害，晚期可达60％，其症状轻者为头痛、头晕、情绪不稳、失眠等，重者可出现抑郁、焦虑、躁狂、智能减退，甚至痴呆，约15％～25％患者有癫痫发作，     

系统性红斑狼疮患者的护理

系统性红斑狼疮是一种弥漫性、全身性自身免疫病，可累及全身多个器官和系统，多发于年轻女性。本病临床表现多种多样，发病可急可缓，早期轻症的患者往往仅有单一系统或器官受累的不典型表现，随着病程的发展其临床表现会越来越复杂，可表现为多个系统和器官受累的临床症状。2011年4月~2013年4月我科收治系统性红斑狼疮患者62例，针对患者不同的临床表现及心理状态予以相应的护理措施，效果良好，现介绍如下。     

核酸识别Toll样受体特性及在系统性红斑狼疮中的作用

系统性红斑狼疮（systemic lupus erythematosus，SLE）是一种由于免疫系统紊乱导致机体产生多种自身抗体的自身免疫病。SLE发病缓慢，临床表现多样化，涉及多个系统和多种脏器损伤，造成细胞和体液免疫功能障碍，产生多种自身抗体。SLE的常见临床表现为发热、皮疹、关节痛、浆膜炎及肾、心血管、肺、神经系统、消化系统等系统损伤，死亡率高。虽然目前SLE发病的确切机制尚不清楚，但对于其发病原因比较公认的是核酸识别Toll样受体对于自身核酸复合物的识别而引发的针对自身的免疫反应。     

狼疮性肾炎的观察及护理

系统性红斑狼疮是一种多发于青年女性、累及多脏器的自身免疫性、炎症性结缔组织病[1].其临床表现复杂,除有常见的皮疹、关节痛、发热、怕光、脱发及多器官或系统损害外,肾脏损害较为突出,表现为血尿、蛋白尿、水肿、高血压、肾功能损害.本病因病情反复,治疗时间长,患者心理负担重,可出现各种心理问题,常常表现为焦虑和抑郁,重者还有自杀倾向,严重影响了患者的治疗和生活.本文通过对狼疮性肾炎的护理经验进行总结,以便采取科学的护理对策,提高患者的治疗依从性.     

1例系统性红斑狼疮多器官系统受累的护理体会

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种弥漫性、全身性自身免疫病,主要累及皮肤黏膜、骨骼肌肉、肾脏及中枢神经系统,同时还可以累及肺、心脏、血液等多个器官和系统,表现出多种临床表现;血清中可检测到多种自身抗体和免疫学异常.2014年11月,我科收治1例多器官系统受累的重症系统性红斑狼疮患者,经积极救治和精心护理,获得较好的效果.现将护理总结如下.     

组织多普勒成像技术对系统性红斑狼疮心脏功能评价的研究进展

目的 探讨组织多普勒成像技术(TDI)对系统性红斑狼疮心脏功能的评价及其进展。方法 通过pubmed数据库、中国知网数据库查阅2009年1月—2015年2月国内外关于组织多普勒成像技术在系统性红斑狼疮心脏病变中的应用有关文献,进行总结归类。结果 发现系统性红斑狼疮心脏病变表现是多方面的,包括心肌、心包、瓣膜以及冠脉等不同程度的损害。这些损害2D超声早期难以判断;而运用TDI相关指标Sm、e′/a′值、e′值、E/e′比值不仅可以对其进行早期评估,还可以对其严重程度及预后可做出准确的判断,从而为临床及时处置提供决策信息。结论 TDI不但能够早期并较好的评价系统性红斑狼疮心脏功能改变,而且对心脏损害的系列病变的检测也具有重要价值。     

系统性红斑狼疮的临床与脑电图

本文对24例系统性红斑狼疮的脑电图结果分析,发现阳性率为45.8％,伴有神经精神症状的阳性率为64.3％。因此,脑电图在评价系统性红斑狼疮所致的脑部损害上是一个较为敏感的客观指标,对狼疮性脑病的早期诊断有较高的实用价值;对系统性红斑狼疮患者的疗效及预后评价也有一定的参考价值。     

系统性红斑狼疮干扰素诱导基因及信号转导途径的研究进展

系统性红斑狼疮（systemic lupusery thematosus,SLE）是一种以大量自身抗体形成为特征,导致多系统、多器官损害的慢性系统性自身免疫性疾病。主要病因可能与遗传、环境因素和雌激素有关,已有研究报道年龄是一大危险因素,尤其以青年女性发病最为常见[1,2]。发病机制主要与免疫调节异常、     

系统性红斑狼疮发病机制相关MicroRNA的研究现状

MicroRNA(miRNA)是新发现的参与高等生物基因表达调控的重要分子,很多新的证据显示miRNA在免疫功能的调控方面占据着举足轻重的地位。系统性红斑狼疮(systemic lupus erythematosus,SLE)是自身免疫性疾病的原型,它以抗核抗体为代表的自身抗体的产生、免疫复合物沉积及多系统损害为特征。SLE的发病机制长期以来是风湿免疫研究领域的难点。本文综述目前所知的与系统性红斑狼疮发病机制相关的microRNA。     

系统性红斑狼疮患者治疗前后凝血、纤溶功能的变化

系统性红斑狼疮（Systemic Lupus Erythematosus,SLE）是一种多器官损害的自身免疫性疾病,常常伴有凝血、纤溶异常,许多临床表现及并发症也与之相关。为进一步认识凝血、纤溶指标在SLE患者中的变化,本文对SLE患者活动期和非活动期及药物治疗前后纤维蛋白原（Fib）、D-二聚体（D-D）、凝血活酶时间的国际标准化比值（PT—INR）、活化部分凝血酶原时间（APTT）和凝血酶时间（TT）等五项指标进行比较,并分析其临床意义。     

A New Model of Induced Experimental Systemic Lupus Erythematosus (SLE) in Pigs and Its Amelioration by Treatment with a Tolerogenic Peptide

Introduction  

Systemic lupus erythematosus (SLE) is characterized by a variety of autoantibodies and systemic clinical manifestations. A tolerogenic peptide, hCDR1, ameliorated lupus manifestations in mice models. The objectives of this study were to induce experimental SLE in pigs and to determine the ability of hCDR1 to immunomodulate the disease manifestations.     

Serum interleukin-17 levels are associated with nephritis in childhood-onset systemic lupus erythematosus

OBJECTIVES:

To determine the serum interleukin-17 (IL-17) levels in childhood-onset systemic lupus erythematosus patients and to evaluate the association between IL-17 and clinical manifestations, disease activity, laboratory findings and treatment.

METHODS:

We included 67 consecutive childhood-onset systemic lupus erythematosus patients [61 women; median age 18 years (range 11-31)], 55 first-degree relatives [50 women; median age 40 years (range 29-52)] and 47 age- and sex-matched healthy controls [42 women; median age 19 years (range 6-30)]. The childhood-onset systemic lupus erythematosus patients were assessed for clinical and laboratory systemic lupus erythematosus manifestations, disease activity [Systemic Lupus Erythematosus Disease Activity Index (SLEDAI)], cumulative damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology (ACR) Damage Index] and current drug use. Serum IL-17 levels were measured by an enzyme-linked immunosorbent assay using commercial kits.

RESULTS:

The median serum IL-17 level was 36.3 (range 17.36-105.92) pg/mL in childhood-onset systemic lupus erythematosus patients and 29.47 (15.16-62.17) pg/mL in healthy controls (p=0.009). We observed an association between serum IL-17 levels and active nephritis (p=0.01) and migraines (p=0.03). Serum IL-17 levels were not associated with disease activity (p=0.32), cumulative damage (p=0.34), or medication use (p=0.63).

CONCLUSION:

IL-17 is increased in childhood-onset systemic lupus erythematosus and may play a role in the pathogenesis of neuropsychiatric and renal manifestations. Longitudinal studies are necessary to determine the role of IL-17 in childhood-onset systemic lupus erythematosus.     

Systemic lupus erythematosus — A state of hyperinterferonemia?

On the basis of immunologic and microbiologic data as well as recent observations it is postulated that interferon is responsible for many of the clinical signs and symptoms in systemic lupus erythematosus such as alopecia, joint manifestations, fever and leukopenia. Recognition of the important role of interferon in active systemic lupus erythematosus leads to some practical conclusions: a) a new therapeutic approach to active lupus must consider a treatment which will lead to reduced interferon activity; b) one of the laboratory parameters of activity of the disease may be interferon measurements.     

Neonatal lupus syndrome: a case with chondrodysplasia punctata and other unusual manifestations.

We report a case of a newborn infant whose mother had systemic lupus erythematosus (SLE) diagnosed before pregnancy. The child had clinical manifestations of neonatal lupus as well as chondrodysplasia punctata and other findings that resemble the congenital anomalies associated with the use of oral anticoagulants, with no history of exposure. We speculate that the combined action of the different maternal autoantibodies may produce the whole spectrum of manifestations.     

系统性红斑狼疮合并肺部感染猝死一例并文献复习

目的：分析系统性红斑狼疮（SLE）的临床特征、治疗及预后,提高医生对SLE合并脏器损伤导致猝死的认识。方法：回顾性分析1例确诊为SLE合并肺部感染猝死的临床特征及治疗过程并结合文献进行复习。以“系统性红斑狼疮猝死”及“Sudden death of systemic lupus erythematosus”为关键词检索1984年1月至2017年2月万方数据库、中国知网及PubMed数据库的相关文献,共获得文献9篇,其中中文2篇,英文7篇。结果：患者女性,26岁,主要临床表现为面部蝶形红斑、发热、胸闷。肾穿刺活检：局灶增生性狼疮性肾炎基底膜增厚,胸部CT示双肺炎症,治疗过程中突然出现室颤而导致死亡。经文献检索共检索到11例SLE猝死患者,5例女性,3例男性,3例性别不详,其中4例资料不全,年龄22~64岁。结论：SLE常合并多系统损伤,当出现心血管症状时,由于常合并其他脏器受累容易被忽视,所以应及时进行心电图、心肌酶、肌钙蛋白等检查,并加强对患者的监护,防止猝死的发生。     

Drug-induced lupus erythematosus

Drug-induced lupus is a syndrome which share symptoms and laboratory characteristics with idiopathic systemic lupus erythematosus (SLE). The terms drug-induced lupus (DIL) and drug-induced lupus erythematosus (DILE) are preferred, but other ones are also used-drug-related lupus, lupus-like syndrome and lupus erythematosus medicamentosus. The first case of DILE was reported in 1945 and associated with sulfadiazine. In 1953, it was reported that DILE was related to the use of hydralazine. More than 80 drugs have been associated with DILE. The average age of patients with DILE is nearly twice that of patients with idiopathic SLE. Approximately half the patients with drug-induced SLE are women, compared with 90% of patients with idiopathic SLE. Similarly to idiopathic lupus, DILE can be divided into systemic, sub-acute cutaneous and chronic cutaneous lupus. The syndrome is characterised by arthralgia, myalgia, pleurisy, rash and fever in association with antinuclear antibodies in the serum. The clinical and laboratory manifestations of drug-induced SLE are similar to those of idiopathic SLE, but central nervous system and renal involvement are rare in DILE. Recognition of DILE is important because it usually reverts within a few weeks after stopping the drug. This review discusses the general issues in DILE, such as pathogenic mechanisms, clinical forms and diagnostic criteria, and provides more detailed information for some of the most recent implicated drugs: minocycline, statins, anti-TNF-alpha agents.     

Recurrent pseudotumor cerebri in systemic lupus erythematosus: a case report.

Pseudotumor cerebri is an uncommon manifestation of neuropsychiatric systemic lupus erythematosus (SLE), and is characterized by an elevated intracranial pressure, papilledema with occasional abducens nerve paresis, absence of a space-occupying lesion or ventricular enlargement, and normal cerebrospinal fluid chemical and hematological constituents. Pseudotumor cerebri has been reported in a few sporadic cases in patients with systemic lupus erythematosus. However, the recurrent pseudotumor cerebri in patients with systemic lupus erythematosus which has been rarely reported, has not been reported in Korea. We experienced a 30-yr-old female patient with SLE who was presented with second attack of severe intractable headache. She was diagnosed pseudotumor cerebri twice and successfully treated with corticosteroid. Headache is the common symptom in patients with neuropsychiatric SLE and attributable to various causes. We suggest that it is important to define the cause of headache in patients with SLE and pseudotumor cerebri should be included in the spectrum of clinical manifestations during the course of SLE as a cause of headache.     

Intravenous immunoglobulin for immunomodulation of systemic lupus erythematosus

Intravenous immunoglobulin (IVIg) is used for replacement therapy in immunodeficiency states and for immunomodulation of various autoimmune diseases. Several case reports and series support a beneficial role of IVIg in systemic lupus erythematosus (SLE), both as salvage immunotherapy and in control of disease activity in general and amelioration of classical disease manifestations. Further, lupus nephritis can also be treated usually successfully with IVIg. A few questions remain unanswered as to the appropriate therapeutic dosage and the clinical manifestations that can be best treated with IVIg.     

Blood-brain barrier disruption and neuroinflammation as pathophysiological mechanisms of the diffuse manifestations of neuropsychiatric systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease that can involve nervous system commitment known as neuropsychiatric systemic lupus erythematosus (NPSLE). The diagnostic of NPSLE is complex because the symptoms range from focal symptoms (e.g., strokes, thrombotic events) to diffuse disorders affecting cognition, mood and level of consciousness (e.g. acute confusional state, psychosis). Both type of manifestations of NPSLE differ in their pathological mechanisms. The focus of this review will be on the mechanisms that lead to the blood-brain barrier (BBB) disruption and to the neuroinflammation related with the diffuse manifestations of NPSLE.