儿童狼疮性肾炎73例临床分析

目的　探讨儿童狼疮性肾炎（ＬＮ）的临床及实验室检查特征。方法　分析７３ 例ＬＮ 患儿的临床表现,统计其发生率、实验室检查结果阳性率,并与１３０ 例成人ＬＮ进行比较。结果　儿童ＬＮ 男女比例为１∶１１;以无症状性血尿和（ 或） 蛋白尿为多发表现;与成人相比肾病综合征并发肾功能损害发生率高。结论　儿童ＬＮ 仍以女性为多见;无症状性蛋白尿和（ 或） 血尿为其常见表现;以肾病综合征为表现时易出现肾功能损害。     

儿童狼疮性肾炎的性别和临床免疫学特征

目的探讨儿童狼疮性肾炎的性别、临床和免疫学特征。方法将71例女性，6例男性红斑狼疮患者的临床、血清免疫学指标和肾脏损害进行比较。结果女性红斑狼疮患者关节炎、白细胞减少比男性常见，而男性盘状红斑较常见，且肾损害比女性严重。结论红斑狼疮临床表现、血清免疫学特征及发病率与性别有关，但男性狼疮性肾炎预后可能较差。     

儿童狼疮性脑病3例

例1，女，7岁，因发热伴浮肿1月，皮疹半月入院，BP12／gkPa，鼻尖、以下肢红色丘疹，眼睑浮肿，肾区叩痛，神经系统检查阴性，Hb67g/L，血小板（PLT）51X109/L，尿红细胞及蛋白均大量，血况（ESR）96mm/1h，C30.41g/L，抗核抗体（ANA）（-），血狼疮细胞（ ），住院第14天出现     

儿童狼疮性肾炎病理分型治疗

狼疮性肾炎(LN)是SLE最重要的临床表现之一,儿童SLE累及肾脏更多见,肾衰竭是SLE主要死亡原因之一.LN的病理分级与疾病活动程度、严重程度及预后密切相关,针对肾脏不同病理分级的治疗已愈来愈成为SLE、LN科学治疗的重要依据.     

儿童狼疮性肾炎分型及治疗策略

儿童狼疮性肾炎（LN）是儿童最常见的肾小球疾病,起病及病情进展均较成人严重,预后较其他肾小球疾病差,影响终生,病因未完全阐明。文章根据最新的国际指南,对儿童LN的病理分型及其相应治疗进行阐述。LN诊断根据2019年EULAR/ACR系统性红斑狼疮分类标准,肾活检病理是目前的诊断的重要手段,增殖型和非增殖型LN治疗分为初始及维持治疗,参照成人指南推荐,合理和积极的治疗对于诱导疾病缓解和避免复发、改善预后非常重要。     

儿童狼疮性肾炎的临床和病理研究

狼疮性肾炎(LN)是决定系统性红斑狼疮(SLE)的临床过程和转归的重要因素之一,其临床表现多种多样,病理改变轻重不一,为探讨儿童LN的临床表现、病理改变及两者之间的关系,现对我院近8年来收治的30例LN患儿进行分析如下。材料与方法一、对象我科1995年12月~2003年6月的住院LN患儿30例,其中男6例,女24例,男∶女为1∶4;起病年龄8~15岁,平均(12.36±1.85)岁,其中<10岁2例;首次就诊前的病程7天~12个月,平均为3.5个月;从确诊到肾活检的时间为3天~2年,肾活检前已行糖皮质激素或免疫抑制剂治疗15例。二、方法1.临床指标:记录所有病例的病程、临床…     

儿童狼疮性肾炎的生物制剂治疗现状

系统性红斑狼疮(SLE)是一种累及多系统、多器官的自身免疫性全身性血管炎疾病。狼疮性肾炎(LN)是儿童SLE最常见、最严重的并发症,是引起我国儿童终末期肾病和儿童死亡的重要病因。随着SLE免疫发病机制研究的不断深入,推动了新型生物制剂治疗LN的研发,极大改善了儿童LN的预后。现对生物制剂治疗LN的现状进行阐述,为临床儿...     

儿童系统性红斑狼疮100例

目的探讨儿童SLE的临床和实验室指标特点及狼疮性肾炎(LN)的肾脏病理类型。方法选取2001年1月-2011年1月本院住院确诊的SLE患儿100例,其中LN 82例,分析其临床表现、实验室和病理检查资料,应用SAS 9.1软件对SLE及各病理类型LN按临床及实验室检查特点行χ2检验。结果 100例SLE患儿,男19例,女81例;发病年龄(10.28±2.98)岁。发病高峰为9～14岁的女性患儿。新生儿1例。临床表现以发热(54%)、皮肤损害(54%)最常见,其次为水肿(34%)、关节炎(22%)、肝脾大(18%)、淋巴结大(15%)、口腔溃疡(4%)。SLE患儿的肾脏累及率为82%。其中肾炎性肾病占33%;血液系统累及率较高,其中贫血59%、低清蛋白血症53%、血小板降低20%;呼吸系统感染40%,肝损害26%,中枢神经受累16%,浆膜炎13%。免疫学检查血清ANA阳性(82%)、补体C4降低(77%)、补体C3降低(70%)、ESR加快(59%)、dsDNA阳性(52%)。48例肾活检病例均有病理异常,病理类型以Ⅳ型最多,占27%;肾脏累及和无肾脏累及组患儿在补体C3降低(P=0.040 9)、抗dsDNA阳性(P=0.023 0)、水肿(P=0.023 6)方面比较差异均有统计学意义。将48例肾活检病例分为增殖性和非增殖性肾炎2组,对Ⅲ、Ⅳ、Ⅴ型+Ⅲ型和Ⅴ型+Ⅳ型(增殖性病变)和Ⅰ、Ⅱ和Ⅴ型(非增殖性病变)进行统计学分析,发现补体C3降低(P=0.042)、抗ANA阳性(P=0.000 6)、ESR加快(P=0.021 9)、贫血(P=0.003 8)差异有统计学意义。结论儿童SLE肾脏、血液系统及呼吸系统累及率最高。补体C3降低、dsDNA阳性和首发表现为水肿的SLE患儿可能更易累及肾脏。肾脏病理表现为增殖性病变的患儿更易发生贫血,补体C3降低、抗ANA阳性、ESR加快提示增殖性病变处于活动期。     

抗心磷脂抗体与儿童系统性红斑狼疮及狼疮性肾炎相关性研究

目的探讨抗心磷脂抗体(ACA)与儿童系统性红斑狼疮(SLE)及狼疮性肾炎(LN)的相关性。方法选取2005年4月至2013年10月住院治疗116例SLE患儿,收集并分析患儿的临床资料。结果 116例SLE患儿中,男20例、女96例;ACA-Ig M、Ig G或Ig A的阳性率分别为37.1%、35.3%、26.7%,阳性率差异无统计学意义(P>0.05)。116例SLE患儿中LN 75例,非LN 41例,LN与非LN患儿之间ACA-Ig M、Ig A阳性率差异无统计学意义(P均>0.05);而LN患儿ACA-Ig G阳性率(42.7%)高于非LN患儿(22.0%),差异有统计学意义(P=0.026)。男性和女性患儿间,LN及ACA各亚型比例的差异无统计学意义(P均>0.05)。结论各亚型ACA对于SLE的诊断价值无差别,但ACA-Ig G阳性提示易并发LN。     

儿童系统性红斑狼疮合并脑后部可逆性脑病综合征4例并文献复习

目的 分析儿童系统性红斑狼疮（SLE）合并脑后部可逆性脑病综合征（PRES）的临床特征,提高对本病的认识。方法 报告北京协和医院诊断的4例儿童SLE合并PRES的临床资料,在PubMed数据库检索相关病例行文献复习,分析儿童SLE合并PRES的临床表现、影像学检查、治疗及预后情况。结果 ①4例SLE患儿均伴有狼疮性肾炎,从确诊SLE至出现PRES的时间1~63个月,出现PRES时1例正在接受甲泼尼龙及环磷酰胺(CTX)冲击治疗。PRES均以惊厥、头痛起病,并伴血压升高。②PubMed数据库检索到11例SLE合并PRES的儿童病例,结合本文报道的4例,15例进入分析。女性14例。年龄最小8岁。从确诊SLE至出现PRES的间隔中位时间为6个月（1个月至8年）。15例出现PRES时均有惊厥发作,10例伴头痛,7例呕吐,9例意识丧失,7例视力障碍。15例均有血压升高。12例有狼疮性肾炎。治疗SLE予甲泼尼龙或CTX冲击治疗分别为4和3例,予羟氯喹2例,予环孢素和利妥昔单抗各1例,PRES分别发生在免疫抑制剂治疗后的2 d至4年。15例行头颅MRI检查示大脑后循环皮质下白质受累为主。12例予降血压治疗,10例予抗惊厥药物短期治疗。9例SLE处于活动期,继予糖皮质激素和免疫抑制剂治疗;6例非活动期的SLE患儿减停糖皮质激素及免疫抑制剂。15例神经系统症状均恢复,随访均未遗留神经系统后遗症。9例复查头颅MRI示颅内病变完全或基本消失。结论 儿童SLE合并PRES主要表现为惊厥、头痛、意识障碍和视觉障碍。对于有狼疮性肾炎的SLE患儿血压升高时,尤其同时予大剂量糖皮质激素或CTX等免疫抑制剂治疗时,应警惕PRES的发生。早期诊断和治疗PRES预后较好。     

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??Objective??To observe the incidence??clinical types??and prognosis of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children??and to probe the relationship between the onset and the relacted factors. Methods??
The clinical data of 87 children with SLE treated in Children’s Hospital of Shanghai from Jan. 2002 to Dec. 2015 were analyzed retrospectively. The patients were divided into NPSLE group and non-NPSLE group. Collect the data of the patients??including the course of the disease??activity index??SLEDAI scores????blood sedimentation??the values of serum complement and the positive rate of dsDNA??anti-ribosomal P-protein autoantibody and anticardiolipin antibody in autoantibodies??and make a comparison and statistical analysis. Results??Nervous system injury occurred in 23 cases??with an incidence rate of 26.4%??and the average course of the disease was 3??11 months. Most of 23 cases had headache. Most of the patients presented SLE activity in serology. Totally 39 cases of all patients had abnormal cranial imaging findings. Electroencephalogram was performed in 21 patients??which presented 12 abnormalities. There were no statistical differences between patients with and without NPSLE in the disease course??blood sedimentation??the values of serum complement??the positive rate of dsDNA or activity index. Clinical symptoms were improved in all 39 patients after treatment??but 2 patients died of heart failure in follow-up. Conclusion??Neuropsychiatric manifestation in children with SLE is common??and it can happen at any stage of the disease. NPSLE in children presents different kinds of symptoms. Early diagnosis and treatment can improve the short-term prognosis. Psychiatric symptoms may also occur during therapy.     

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??Objective??To analyse the positron emission computed tomography??PET/CT?? findings of neuropsychiatric systemic lupus erythematosus??NPSLE?? in children. Methods??Patients diagnosed with NPSLE treated at Department of Pediatrics??Peking Union Medical College Hospital from April 2012 to February 2014 were recruited. PET/CT using F-18-labelled fluorodeoxyglucose??18F-FDG?? was performed in patients with NPSLE??and the results were analyzed. Results??A total of 19 patients received 18F-FDG PET/CT and 13 of them were females. Age was from 7to14 years and median age was 11.3 years. The most common neuropsychiatric manifestation was headache??8/19???? followed by seizure??7/19???? cognition dysfunction??2/19????psychiatric disease??1/19?? and ataxia??1/19??. All patients under MRI examination??and only 4 of them were abnormal. 18F-FDG PET/CT revealed significant glucose hypometabolism in all patients. The temporal lobe??17/19?? was the most susceptible brain regions??followed by the parietal lobe??16/19????occipital lobe??14/19?? and frontallobe??10/19??. In contrast??glucose hypermetabolism was detected in basal ganglia??8 patients?? and thalamus??3 patients??. Conclusion??PET reveals significant glucose hypometabolism with or without local hypermetabolism in patients with NPSLE.     

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??Objective The aim of this study was to summarize the clinical features in children with neuropsychiatric systemic lupus erythematosus??NPSLE?? and to investigate the related risk factors of in systemic lupus erythematosus??SLE?? patients with NPSLE. Methods The clinical data of 14 children with NPSLE were retrospectively analyzed. The indicators including the age?? sex?? clinical manifestations?? laboratory tests were compared with 31 randomly selected SLE patients with non-NPSLE manifestations by univariate and multivariate analysis. Results The main clinical manifestations in children with NPSLE were headache??50%????lateral type motor disorder??50%????mental disorders??35.7%????epilepsy??35.7%????involuntary movement ??14.29%????blurred vision??14.29%?? etc. 13 patients had abnormalities in brain MRI. Univariate analysis showed that children with oral ulcers??blood lymphopenia??thrombocytopenia??elevated CRP??anticardiolipin antibodies positive??antineutrophil cytoplasmic antibody positive??anti-u1-RNP antibody positive??urine red blood cells increased??elevated serum K+ elevated??blood urea nitrogen??elevated serum creatinine were the risk factors with statistical significance. Further non-selective multivariate regression analysis showed that only the anticardiolipin antibody positive?? elevated CRP?? elevated serum creatinine into the multiple regression equation?? a positive correlation. Conclusion Headache?? movement disorders?? mental disorders and seizures were the most common symptoms in children with NPSLE. The brain MRI is a sensitive indicator of checking NPSLE. Anticardiolipin antibody positive?? elevated CRP and elevated serum creatinine were the most important risk factor for SLE children with NPSLE.     

小儿系统性红斑狼疮的特点

目的了解小儿系统性红斑狼疮(SLE)的特点。 方法对85例SLE儿的临床资料进行分析。 结果85例中起病年龄10a以上者66例(77．6％),男女比为16．1,12例(14．1％)家族中有结缔组织病史。最常见的表现是抗核抗体(ANA)阳性(91．8％)、血沉增快(90．6％)、肾脏受累(82．3％)、发热(82．3％)、低补体血症(81．2％)、蝶形红斑(69．4％)、关节症状(62．3％)及血液系统损害(62．3％)。18例(21．2％)起病时表现为单一系统损害。6例(7．0％)肾损害始终为唯一临床表现。经激素联合免疫抑制剂治疗,随访0．5～23a,临床缓解或病情波动者49例,无1例进入慢性肾功能衰竭,死亡9例,失访27例。 结论本病临床表现多样,肾损害发生率高,早期正规治疗疗效显著。     

儿童神经精神性狼疮临床特征及危险因素分析

目的总结儿童神经精神性狼疮(NPSLE)的临床特征及危险因素。方法回顾分析2013年1月至2018年12月住院治疗的SLE患儿的临床资料。结果纳入315例SLE患儿中,58例确诊NPSLE,男11例、女47例,平均年龄(10.45±2.63)岁;共出现14种神经精神症状,依次为头痛21例、癫痫发作19例、脑血管意外15例、脱髓鞘综合征13例、情绪失调10例。58例NPSLE患儿均行头颅影像学检查,异常53例(91.4%);44例行脑电图检查,异常38例(86.4%);39例行脑脊液检查,异常34例(87.2%)。首次发病发生NPSLE较未发生NPSLE的患儿,血小板计数100×10~9/L的发生率高,差异有统计学意义(P0.05)。2013年1月至2015年12月发病的23例患儿病死率为21.7%,高于2016年1月至2018年12月发病的35例患儿(病死率2.9%),差异有统计学意义(P0.05)。多元logistic回归分析表明,血小板计数降低、抗心磷脂抗体阳性、激素治疗不规范是发生NPSLE的独立危险因素(P0.05)。结论 NPSLE以头痛、癫痫发作最多见,血小板计数降低的NPSLE患儿更早出现神经精神症状。近年NPSLE患儿病死率较前有所降低。血小板计数降低、抗心磷脂抗体阳性、激素治疗不规范是SLE患儿发生NPSLE的危险因素。     

Antineuronal antibodies and electroneurophysiological studies in pediatric patients with neuropsychiatric systemic lupus erythematosus

Neuropsychiatric systemic lupus erythematosus (NPSLE) is one of the most difficult manifestations of lupus to diagnose. Measurement of serum brain autoantibodies and assessment of cognitive function by electroneurophysiological studies (electroencephalogram (EEG) and P300) have contributed to an earlier and a more specific diagnosis of NPSLE. Thus, we were stimulated to assess the value of serum antineuronal antibodies and electroneurophysiological studies in diagnosis and early prediction of NPSLE. To investigate this, assessment of serum antineuronal antibodies and cognitive function (clinically and by electroneurophysiological studies) was done in 30 lupus patients [14 (46.7%) with and 16 (53.3%) without clinical evidence of NPSLE] in comparison with 30 healthy matched subjects. Patients without clinical evidence of NPSLE were followed-up clinically by monthly neuropsychiatric evaluation for 18 months. Seropositivity for antineuronal antibodies and abnormalities of EEG and P300 (prolonged latency and/or low amplitude) were found in 60%, 50% and 70%, respectively of lupus patients. During follow-up, 8 out of the 16 patients without clinical evidence of NPSLE developed such evidence [six (75%) had antineuronal seropositivity, five (62.5%) had abnormal EEG, six (75%) had P300 abnormalities and all had at least one abnormal result of these parameters at the time of initial evaluation before clinical presentation of NPSLE]. In conclusion, serum antineuronal antibodies and electroneurophysiological studies may be reliable parameters for diagnosis and early prediction of NPSLE, especially when combined together, before clinical manifestations ensue. Further studies on a large scale are warranted to evaluate the predictive value of these parameters in NPSLE.     

儿童神经精神性狼疮的发病机制及诊治进展

儿童神经精神性狼疮(neuropsychiatric systemic lupus erythematosus,NPSLE)是系统性红斑狼疮(systemic lupus erythematosus,SLE)的严重并发症,发生率高达95％.目前认为NPSLE发病机制是多种因素作用的结果,包括血脑屏障破坏、血管栓塞、神经内分泌功能紊乱、自身抗体的产生以及炎性介质的参与.临床表现复杂多样,主要表现为头痛、癫痫样发作及精神障碍.国际上尚无统一的诊断标准,必须综合应用免疫血清学、脑脊液检测、神经影像学和神经精神的评估,治疗主要应用免疫抑制剂、生物治疗和对症治疗,早期诊断和规范治疗有助于改善预后.     

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??Abstract??Objective??To observe the efficacy and safety of Rituximab ??Roche Pharmaceuticals RTX?? trade name?? rituximab?? treatment for children with systemic lupus erythematosus??and try to find a useful alternative therapeutic approach to those with bad response to traditional therapies. Methods??Produced by Roche Pharmaceuticals??CD20 monoclonal antibody rituximab was used by intravenous injection. Initial dose was 188 mg/m2?? 2 weeks later the second injection was given at 375 mg/m2. Thirty minutes before each injection 5mg Dexamethasone and 10mg Promethazine were given to prevent drug allergy. Results??Twelve cases of SLE in children were performed flow cytometry detection of CD20+ 4 weeks after administration??10 cases were 0??85.71%?? in B cell depletion?? and gradually rose after 6??8 months. IgG?? IgM and IgA plasma had no significant difference from the baseline. Clinical manifestations and clinical signs in 12 cases of children were evaluated??and the average score dropped from 16.0±2.95 to 8.67±1.83. ANA?? Anti-ds-DNA?? C3 and C4 had different degrees of improvement. In 6 cases of lupus nephritis children with urinary protein was significantly improved after 6 months of Rituximab treatment??4 cases of elevated serum creatinine and blood urea nitrogen was also returned to normal after 6 months of treatment. One case had severe pulmonary infection. Conclusion??Significant effects of CD20+ monoclonal antibody ??rituximab?? treatment are shown for children with SLE and lupus nephritis?? especially in severe children. This provides a new alternative treatment for those with poor tolerance to traditional Prednisone and cyclophosphamide treatment and with poor clinical effect. However?? infection problem can not be ignored. The Rituximab replacement treatment for children with SLE still need further study.     

A 10 year review of systemic lupus erythematosus in Singapore children

A 10 year retrospective analysis of the clinical features and survival of 24 Singapore children with systemic lupus erythematosus was made. The female to male sex ratio was 11:1. The median age at diagnosis was 9.3 years (range: 3.5–17.6 years), and the median duration of follow-up was 3.6 years (range: 3 months - 10 years). The common modes of presentation were prolonged fever and malar rash (both 46%). Renal involvement (71 %) was frequent. There were six deaths, three from chronic renal failure, two from infection, and one from carditis. The overall survival at 5 years was 0.800 (s.e.m. = 0.090), and at 10 years 0.698 (s.e.m. = 0.103). The survival for lupus nephritis was 0.727 at 5 years (s.e.m. = 0.116), and 0.586 at 10 years (s.e.m. = 0.130). Although the 5 year survival rate is comparable with other series, there were more deaths after the first 5 years, and morbidity from the disease as well as from therapy was considerable.