儿童狼疮中枢神经系统的急症表现

目的：探讨儿童系统性红斑狼疮中枢审经系统急症的特征,方法：将狼疮脑病患儿与继发中枢神经系统损害狼疮患儿的ANA,dsDNA滴度,Sm阳性率及补体C3下降的阳性率进行比较,并对两组患儿的临床特征进行分析,结果：ANA、ds-DNA滴度及Sm阳性率,补体C3下降阳性率与狼疮脑病并不相关,脑电图有助于狼疮脑病的诊断。结论：儿童红斑狼疮中枢急症原发及继发性损害的鉴别诊断需结合临床及有关辅助检查综合分析。     

风湿病及结缔组织病

932138血清抗EVA抗体在儿童风湿性疾病中的意义/陈燕…//实用儿科临床杂志一1993,8(2)一82一83 对56例结缔组织病患儿常规进行抗核抗体(ANA)及抗DNA抗体测定,随机取样进行抗ENA抗体分析.结果:抗ENA抗体仅特异性存在儿童系统性红斑狼疮(SLE)及混合结缔组织病(MCTD)病人血清中,SLE阳性例数24/31例,MCTD为2/2例,余均阴性.在抗ENA抗体阳性的SLE病例中,抗Sm、RNP屯ssA、SSB抗体阳性率分别为87.5%,62.5%,65%和65%。血清抗ENA抗体的滴度,与ANA滴度不一致,而与荧光抗核抗体(FANA)的图型有关。出现狼疮脑炎的患儿,FANA滴度与…     

系统性红斑狼疮心脏损害及其相关因素分析

目的　探讨系统性红斑狼疮 (SLE)并心脏损害的特点及相关因素。方法　对 78例SLE患儿进行回顾性分析 ,并分为并心脏损害组 (40例 )与无心脏损害组 (38例 )。结果　SLE患儿并心脏损害占 5 1.3% ,最常见为心包积液 ,占 33.3% ;其次心肌炎 ,占 2 1.8% ;肺动脉高压占 12 .8%。SLE并心脏损害组与无心脏损害组在发热、高血压、肾功能不全、高滴度ANA、抗DNA阳性及低补体C3 方面均有显著差异 (P均 <0 .0 5 ) ,并得出其线性关系 :^ Y=- 8.4 32 0 .2 5 9T 0 .2 1BUN 5 .3× 10 -5ANA 1× 10 -4DNA - 0 .137C3 ,r =0 .76 2。结论　SLE心脏损害是与否发热、高血压、肾功能不全、高滴度ANA、抗DNA阳性及低补体C3 有关 ,SLE心脏损害提示病情较重     

抗核小体抗体在儿童系统性红斑狼疮中的诊断意义

目的 了解儿童系统性红斑狼疮(systemic lupus erythematosus,SLE)临床特点,探讨抗核小体抗体(anti-nucleosome antibodies,AnuA)与SLE临床特点的关系.方法 回顾性分析2011年4月至2017年4月在江西省儿童医院住院确诊的SLE患儿病历资料.结果 58例SLE患儿中,29例AnuA阳性与29例AnuA阴性SLE患儿比较,两组间水肿、贫血、肾功能、狼疮性肾炎的病理活动性指数、慢性指数、抗dsDNA抗体阳性率、抗组蛋白抗体阳性率、抗心磷脂抗体阳性率和补体C4下降程度等指标差异均有统计学意义.AnuA阳性SLE患儿肾脏损害程度更重,且病理损害活动度更高,抗dsDNA抗体阳性率、抗组蛋白抗体阳性率、抗心磷脂抗体阳性率更高,补体C4下降程度和贫血程度更重.结论 AnuA对SLE的诊断具有一定指导意义,并可作为SLE患儿进行肾活检的参考指标之一.     

DNA免疫吸附治疗儿童重症狼疮近期疗效及安全性分析

目的 探讨DNA免疫吸附治疗儿童重症狼疮的近期临床疗效和安全性.方法 2007年4月至2008年7月中国医科大学附属盛京医院小儿肾脏内科对9例重症狼疮患儿采用血液灌流DNA免疫吸附柱对每例进行3次吸附.观察其临床表现、对ANA、ds-DNA等自身抗体的清除效果,对免疫球蛋白、血清补体、24h尿蛋白定量、血常规、肝、肾功能、心肌酶谱、血清离子、血沉的影响.结果 DNA免疫吸附后患儿的症状和体征均明显改善;ANA抗体滴度吸附后明显下降[(2515.56±1772.62)及(477.89±272.31),P＜0.001];抗ds-DNA抗体被完全清除,其它自身抗体也被清除;免疫球蛋白IgG吸附后下降明显[(14.47±5.07)g/L及(8.53±1.30]g/L,P＜0.001];血清补体有上升趋势,24h尿蛋白有下降趋势;时肝、肾功能、心肌酶谱、血清离子、血沉无明显影响,有4例血小板下降.结论 DNA免疫吸附疗法能够在短时间内清除重症狼疮患儿体内抗ds-DNA和ANA抗体等免疫物质,使患儿度过免疫风暴导致的危重期,且具有较好的安全性.     

儿童狼疮脑病39例报告

为探讨儿童狼疮脑病的临床特点及预后，通过长期随访，对１３０例系统性红斑狼疮（ＳＬＥ）患儿中的３９例狼疮脑病的临床资料进行了分析和总结。结果，狼疮脑病的发生率为３０％。其中８５％（３３／３９）的患儿在确诊ＳＬＥ第１年内出现中枢神经系统（ＣＮＳ）受损，１８％（７／３９）以ＣＮＳ损害作为ＳＬＥ首发症状。最常见ＣＮＳ异常是癫痫发作、头痛、昏迷、急性器质性脑病综合征样表现。随访的２５例中，坚持按医嘱服泼尼松者１２例，病情持续稳定；间断服药、自行加减泼尼松剂量１１例，病情波动，其中６例间断发热、皮疹、关节肿痛，５例遗留ＣＮＳ后遗症；自行停止治疗的２例均死亡。提示，ＣＮＳ受累是儿童ＳＬＥ常见的严重并发症，临床表现具多样性，故应注意鉴别诊断。早期诊断和治疗，长期随访和坚持按医嘱服药，可望获得良好预后。     

以假性肠梗阻为首发表现的儿童隐匿型狼疮1例报道并文献复习

目的分析以假性肠梗阻(IPO)为首发表现的儿童系统性红斑狼疮(SLE)的临床特点。方法回顾性分析我院1例以IPO为首发表现的儿童SLE的临床特点及诊治经过,并检索国内外相关文献进行复习、总结。结果该例患儿临床主要表现为反复发作的呕吐、腹痛,逐渐加重,X线腹平片显示不完全性肠梗阻,同时有浆膜炎、血液系统及肾脏受累表现,ANA、抗dsDNA抗体、抗RNP抗体、抗SSA抗体、抗SSB抗体均阳性,补体C3、C4下降。经糖皮质激素治疗后病情很快缓解。结论 IPO是SLE的一种少见的、临床上缺乏足够认识的合并症,而以IPO首发的SLE更为少见,极易误诊。应尽量做到早期诊断,合理治疗,以避免不必要的手术及不良预后。     

儿童系统性红斑狼疮神经精神表现研究

目的观察系统性红斑狼疮患儿神经精神表现发生率、临床类型以及预后,探讨其发生与相关因素的关系。方法回顾性分析2002年1月至2015年12月上海市儿童医院收治的87例系统性红斑狼疮患儿临床资料,患儿分为有神经精神表现的神经精神性狼疮(NPSLE)组与非NPSLE组,对所有SLE患儿病程、活动指数(SLEDAI评分)、红细胞沉降率、血清补体值、自身抗体中抗双链抗体、抗核糖体P蛋白抗体和抗心磷脂抗体阳性率等指标进行比较并作统计学分析。结果 87例系统性红斑狼疮患儿中出现神经精神表现的NPSLE患儿23例,发生率为26.4%,病程3~11个月。23例NPSLE中以头痛症状为主。大部分血清学均呈狼疮活动表现。87例SLE患儿中有39例头颅影像学检查异常。21例行脑电图检查,12例异常。NPSLE组与非NPSLE组病程、红细胞沉降率、补体、自身抗体阳性率、活动指数等差异无统计学意义。23例经治疗短期预后良好,但在随访中有2例因心衰死亡。结论儿童NPSLE表现各异,其可发生在疾病非活动期在内的任何阶段,早期发现并及时积极治疗可改善短期预后。在相关药物治疗过程中也可能出现精神症状。     

儿童神经精神性狼疮12例影像学表现及临床分析

目的 探讨儿童神经精神性狼疮的临床特点与影像学的关系.方法 回顾性分析我院小儿肾脏风湿免疫科2005年1月至2013年1月间收治的12例确诊儿童神经精神性狼疮患儿的临床资料及影像学资料.结果 12例患儿均处于狼疮重度活动期,均行头部MRI或CT检查.3例行头部CT检查,2例异常;11例行头部MRI检查,10例发现异常,弥漫型病变4例,病变主要分布在双侧大脑半球、基底节区、半卵圆中心及小脑半球,多发点、片状T1低信号、T2高信号影,FLAIR与DWI表现为高信号或等信号;局灶型病变4例,表现为单一部位的T1低信号、T2高信号,FLAIR及DWI表现为相同部位高信号;病变部位在大脑半球2例、脑干1例、小脑1例.脑萎缩病变1例,脑出血病变1例.结论 头部MRI诊断儿童神经精神性狼疮敏感性高,抗心磷脂抗体阳性可能与系统性红斑狼疮中枢神经系统损害有密切关系.狼疮重度活动时需警惕神经精神性狼疮的发生,结合患儿病史特点、临床表现及影像学改变,除外其他原因引起的神经系统症状,早期诊断及积极治疗,患者预后可能良好.     

狼疮性肺炎4例

目的总结儿童狼疮性肺炎的临床特点,帮助早期诊断及改善预后。方法回顾性分析本院2010年1月-2011年12月收治的35例SLE患儿中4例并狼疮性肺炎患儿临床资料。4例均为女童,年龄5～11(8.2±2.3)岁,体质量(24.8±5.7)kg。比较其临床表现有无差异,记录狼疮性肺炎患儿ESR、CRP,降钙素原、补体C3和C4水平及血培养、痰培养结果,G试验、GM试验等指标,分析4例患儿胸部CT表现。4例患儿均予激素、环磷酰胺治疗。结果 4例患儿均有发热、咳嗽、气促、呼吸困难,三凹征阳性,肺部影像学改变,ESR、CRP、降钙素原均明显高于正常值,痰培养、血培养均为阴性,G试验、GM试验结果为阴性。给予环磷酰胺联合糖皮质激素治疗,4例均治愈。结论儿童狼疮性肺炎临床表现缺乏特异性,可结合影像学及实验室检查协助诊断,环磷酰胺联合糖皮质激素治疗有效。     

Rhupus arthropathy as the presenting manifestation in Juvenile SLE: a case report

An 8.5-year-old girl was referred with swelling of both knees lasting for two years. ANA was found as negative. She was diagnosed as oligoarticular JIA. After two years of follow-up, thrombocytopenia was detected during routine screening. Her ANA and anti ds-DNA antibodies also became positive, with low levels of C3 and C4. She was diagnosed as Juvenile SLE, meeting the criteria cytopenia, positive immunoserology (anti dsDNA), positive ANA test, and four years of ongoing chronic arthritis, so called as "rhupus arthropathy". We should be aware of the several initial incomplete presentations of lupus in children. We should be careful in monitoring the serious manifestations of the disease in juvenile lupus patients with rhupus arthropathy, and consider the poor response to standard disease modifying agents.     

Differential manifestations of prepubescent, pubescent and postpubescent pediatric patients with systemic lupus erythematosus: A retrospective study of 96 Chinese children and adolescents

Background

Children represent 10-20% of all systemic lupus erythematosus (SLE) patients. Their clinical manifestations and outcomes vary with age. We aim to clarify the relationship between pubescent status and the clinical manifestations of pediatric SLE.

Methods

In this study, pediatric SLE patients were divided into three groups, based on age at disease onset (?Q8, 8?C13 &; 13?C18?years), defined as prepubescent, pubescent and postpubescent, respectively. Initial clinical manifestations and laboratory characteristics at diagnosis were analyzed.

Results

Ninety-six patients were entered into the study: 8 had disease onset before age 8, while 49 were between 8?C13 and 39 of them were 13?C18. Female predominance was noted in all three groups (2.5-7.0:1). Postpubescents showed significantly more renal involvement and lymphopenia, along with lower levels of C3 and C4, when compared with prepubescents. They also showed significantly more lymphopenia when compared with pubescents. Pubescents showed significantly more renal involvement, leukopenia and lupus anticoagulant (LAC) positivity, along with lower C3 and C4 levels, when compared with prepubescents. Pubescents also showed significantly higher anti-Sm antibody positivity when compared with postpubescents. Prepubescents showed significantly more splenomegaly and anti-Jo-1 antibody positivity when compared with those of pubescents. The results showed that the disease activity (SLEDAI-2K score) correlated positively with age at disease onset and negatively with disease duration before diagnosis (p?=?0.011).

Conclusions

Age at disease onset is related to initial manifestations in pediatric SLE patients at our center. Certain parameters such as renal involvement, splenomegaly, low C3 level, low C4 level, lymphopenia, leukopenia, and anti-Sm &; anti-Jo-1 antibody were found to be significantly different among the age groups. Renal involvement might be the key symptom that varies with age.     

Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study

OBJECTIVE: To describe the clinical and laboratory manifestations of childhood-onset systemic lupus erythematosus (SLE) at presentation. STUDY DESIGN: This retrospective French multicenter study involved 155 patients in whom SLE developed before the age of 16 years. Mean patient age at onset was 11.5 +/- 2.5 years (range, 1.5-16 years). The female to male ratio was 4.5. RESULTS: The most common initial manifestations were hematologic (72%), cutaneous (70%), musculoskeletal (64%), renal (50%), and fever (58%). Thirty-two percent of children had atypical symptoms, mainly including abdominal involvement in 26 patients, which lead to negative laparotomy results for presumed appendicitis. Severe renal, neurologic, hematologic, abdominal, cardiac, pulmonary, thrombotic, and/or cutaneous manifestations occurred within the first month after the diagnosis in 40% of patients. The mean erythrocyte sedimentation rate was 72 +/- 29 mm/h, and the mean C-reactive protein value 22 +/- 21 mg/L. Antinuclear antibodies an, anti-double stranded DNA antibodies, and low C3 or C4 level were retrieved in 97%, 93%, and 78 % of patients, respectively. CONCLUSION: Initial manifestations of childhood-onset SLE are diverse and often severe. The diagnosis of SLE should be promptly considered in any febrile adolescent with unexplained organ involvement, especially when associated with an increased erythrocyte sedimentation rate.     

Childhood lupus: Experience from Eastern India

Objective

To delineate the clinical behavior of SLE in children from Eastern India and to the differences in disease pattern.

Methods

In the present study, all 44 patients of pediatric SLE who were diagnosed over a period of 5 years in our pediatric rheumatology clinic were followed prospectively. The resultant database was analyzed using standard statistical methods.

Results|About 3.9% of all rheumatology cases dealt with in the clinic in the last five yrs (n=1063) were SLE. The number of children in 5–8 yrs and 8–12 yrs age groups were 13 and 27, respectively. The overall female (n=35) to male (n=9) ratio in this study was 3.8:1. Renal, hematological and Neuropsychiatric features were most common major organ manifestations(54%,54% and 25% respectively). Joints and skin were the most common minor organs involved. Two case were ANA negative SLE. Among the typical features of ANA negative disease, only nephritis was found in these patients. Anti dsDNA was positive in 50 % cases (n=21). C3 levels were studied in all cases with nephritis (n=22) and 68 % (n=15) had hypocomplementemia. Anti Ro and anti La antibodies were positive in two cases of neonatal lupus. APLA, Anti Sm antibody and anti U1RNP were negative in the cases where testing was done

Conclusions

This study has tried to delineate the disease trends of childhood lupus from Eastern India. Certain important trends have emerged which are different from other contemporary Indian and International observations.

     

Childhood systemic lupus erythematosus in South India

Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F =1∶4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, I due to cardiac and 2 due to central nervous system involvement.     

Sjogren syndrome in childhood: report of two cases

A 12-year-old boy who developed primary Sjogren syndrome and a girl, whose diagnosis of secondary Sjogren syndrome was established at the age of 3, are reported. The importance of some unusual manifestations at disease onset and possible differences in expression between children and adults are briefly discussed.Abbreviations ANA antinuclear antibodies - RF rheumatoid factor - SS Sjogren syndrome - 1°SS primary Sjogren syndrome - 2°SS secondary Sjogren syndrome     

Systemischer Lupus erythematodes im Kindesalter

The clinical manifestations of systemic lupus erythematosus (SLE) are heterogeneous. Non-specific symptoms such as fever, fatigue, lymph node swelling and loss of weight may precede systemic organ manifestations, making an early diagnosis difficult. Skin manifestations like the classical butterfly rash, as well as involvement of joints, kidneys, the central nervous system, and hematopoiesis are common and characteristic. Helpful laboratory results are an increased ESR, low complement factors such as C4 (hypocomplementemia) and, in particular, antibodies against double-stranded DNA. This article summarizes the clinical manifestations and the most important steps in diagnosing SLE in children and adolescents.     

Antinuclear antibodies and lupus-like syndromes in children receiving anticonvulsants.

Drug-induced systemic lupus erythematosus (SLE)-like syndromes in children are most commonly associated with the administration of ethosuximide, diphenylhydantoin, and trimethadione. Five children receiving ethosuximide who presented with syndromes suggestive of SLE were studied. Each and fever, malar rash, arthritis, and lymphadenopathy. Two children had pleural effusions and another developed myocarditis and pericarditis. Three patients had anti-DNA antibodies associated with low serum C3. In four of five children symptoms disappeared with the discontinuation of ethosuximide; two of these continue to have antinuclear antibodies (ANA). One child continues to have active SLE with nephritis. A group of 101 children from a seizure clinic were tested for the presence of ANA. ANA were found in 14 of 70 children receiving ethosuximide and/or diphenylhydantoin; 2 of 14 had anti-DNA antibodies. Serum ANA titers in the drug-induced SLE group did not differ significantly from those of the asymptomatic seizure patients. ANA were also present in 5 of 23 children receiving phenobarbital only. The induction of ANA by phenobarbital is a possible hypothesis. Quantitative immunoglobulins and C3 were not significantly altered in the asymptomatic children with ANA. Follow-up studies at ten months showed no asymptomatic child with ANA to have developed clinical with ANA to have developed clinical evidence of SLE. This study suggests that asymptomatic children who develop ANA should have careful observation, but need not have their anticonvulsants discontinued.     

不同性别系统性红斑狼疮患儿的临床特征及随访比较

目的 对不同性别系统性红斑狼疮（SLE）患儿的临床特征及随访情况进行比较。方法 回顾性分析2008~2018年确诊为SLE的≤14岁患儿79例（男18例,女61例）的临床资料,对男女儿童首发及主要临床症状、器官/系统损害情况、相关实验室指标、随访情况等进行对比研究。结果 首发症状及非首发症状中,男童组以发热发生率最高,女童组以面部红斑发生率最高。男童更易出现肾脏和血液系统的损害（P < 0.05）,其中蛋白尿发生率较女童明显升高（P < 0.05）。女童更易出现关节痛（P < 0.05）。两组抗核抗体、抗双链DNA抗体、补体C3、红细胞沉降率的异常率均很高（>80%,P > 0.05）。男童组的疾病活动度在首次就诊及随访至第9年时高于女童组（P < 0.05）。随访结果显示（随访时间1个月至10年）,在男童组中,3例失访,1例死亡,7例病情控制良好,但均需口服较大剂量激素或免疫抑制控制病情,2例发展为慢性肾功能衰竭,1例出现狼疮脑病。女童组中,3例失访,5例死亡,34例病情控制良好,其中口服醋酸泼尼松10 mg以下维持的有5例,停药1年的1例,停药2年的2例;4例出现狼疮脑病;1例在患病后第7年出现抑郁焦虑状态并有自杀倾向;2例出现视力下降、视物模糊、绿视;1例在服用激素第3年出现双侧股骨头缺血坏死。结论 不同性别SLE患儿的临床特征、部分实验室指标及预后存在差异。男性SLE患儿可能起病更重,更易发生肾脏及血液系统损害,且远期预后可能更差。女性SLE患儿可能更易累及关节。     

Neuropsychiatric involvement in juvenile systemic lupus erythematosus

Neuropsychiatric involvement is an important cause of morbidity and mortality in systemic lupus erythematosus (SLE) and it has been reported to occur in 22-95% of the childhood SLE patients. The aim of this study was to evaluate the neuropsychiatric involvement in our juvenile SLE patients. This was a cross-sectional assessment of patients to investigate the relationship between the involvement of the nervous system and the clinical factors, including autoantibodies, renal involvement and disease activity. We used Symptom Checklist-90-R (SCL-90-R), designed to measure the psychopathological symptoms. As controls, we used 20 healthy adolescents and 20 patients with chronic diseases without any neuropsychiatric manifestations. Overall, 55% (n= 11) of the patients displayed neurological symptoms and/or signs. However, central nervous system (CNS) imaging showed pathological findings only in four of these patients. Patients with headache only had normal CNS imaging. Nine patients had moderate to severe depression. When SLE patients were compared to healthy controls and to adolescents with chronic diseases, they were found to be significantly more depressed. In conclusion, pediatric rheumatologists should be aware of the frequency of neuropsychiatric disturbances in SLE. The neuropsychiatric disorders do not always correlate with disease activity and these children need professional psychological evaluation.