Juvenile rheumatoid arthritis in India—rarity of antinuclear antibody and uveitis

Juvenile chronic arthritis is a heterogenous disease, having different subtypes. Among our 89 patients with juvenile chronic arthritis, we did not find even one patient with early onset pauciarticular disease with uveitis and antinuclear antibody positivity. Further, the prevalence of anti-nuclear antibodies and anti-histone antibodies was very low whereas the prevalence of rheumatoid factor was similar to that reported from Western countries. Thus, the spectrum of juvenile chronic arthritis in India differs from that seen in the west. Larger population based studies of the disease are thus needed.     

生物制剂在儿童风湿免疫性疾病中的应用

生物制剂开辟了靶向治疗风湿免疫性疾病的先河,为经传统抗风湿药物治疗无效的风湿免疫性疾病儿童提供了新的选择.本文介绍了细胞因子拮抗剂及细胞靶向生物制剂的种类、用法、疗效、不良反应及注意事项,为指导临床合理用药提供参考.     

Clinical and ultrasound assessment of the knee in children with juvenile rheumatoid arthritis

Objective  To correlate clinical features with ultrasound (USG) findings in the detection, quantification and follow up of inflammatory signs of knee in children with mono or pauciarticular juvenile rheumatoid arthritis (JRA). Methods  Thirty patients (11 girls, 19 boys) with pauciarticular JRA (14 with monoarticular and 16 with bilateral knee involvement) were studied. Mean disease duration was 10 months (range 2 months to 5 yr). All knees were classified into two groups, according to the presence or absence of acute inflammation. Clinical assessment and ultrasound was done in all patients on the same day. All the patients received naproxen (15–20 mg/Kg/day) for a period of six months, after which clinical assessment and ultrasound study was repeated. Results  Synovial proliferation and effusion, was demonstrated in a much higher frequency in those clinically active (Group A) as compared to these in clinical remission (Group B). Statistically significant differences between clinical and USG indices were seen. Conclusion  USG of knee is more sensitive than clinical assessment in detection of synovial effusion and thickening and plays a useful role in monitoring evolution of the inflammatory process, its quantification and for follow up.     

CD28表达与幼年特发性关节炎发病的相关性研究

目的 探讨CD28在幼年特发性关节炎(juvenile idiopathic arthritis,JIA)活动期及疾病静息期变化的意义.方法 利用流式细胞仪技术检测36例初发JIA患儿治疗前和疾病稳定后外周血CD4+、CD8+T细胞表面CD28的表达.结果 JIA患儿疾病活动期外周血CD4+T细胞CD28+表达显著低于正常对照组(P＜0.01),CD8+T细胞CD28+表达显著低于正常对照组(P ＜0.01);JIA患儿疾病活动期外周血CD28-表达的CD4+T细胞显著高于正常对照组(P ＜0.01);JIA患儿疾病活动期外周血CD4+T细胞数量显著高于正常对照组,CD8+T细胞显著减少(P ＜0.01);JIA患儿疾病静息期外周血CD4+T细胞和CD8+T细胞数量与正常对照组差异无统计学意义(P＞0.05);JIA患儿疾病静息期外周血CD4+T细胞CD28表达和CD8 +T细胞CD28表达与正常对照组差异无统计学意义(P＞0.05).结论 CD28-的表达水平可以作为JIA疾病活动期的指标之一;JIA患儿疾病活动期CD4+T细胞、CD4+ CD28-T细胞出现凋亡障碍.     

Diabetes, coeliac disease, multiple sclerosis and chronic arthritis in first-degree relatives of patients with juvenile idiopathic arthritis

Aim: To evaluate the occurrence of autoimmune diseases in first‐degree relatives of children with juvenile idiopathic arthritis (JIA) and to compare the figures with published population data. Materials and methods: Families of the 362 children with recently diagnosed JIA admitted to Rheumatism Foundation Hospital, Finland, from 1996 to 2001 were contacted by questionnaires regarding autoimmune diseases in family members. Data were collected on type 1 diabetes, coeliac disease, multiple sclerosis and chronic arthritis, consisting mainly of JIA, rheumatoid arthritis, spondyloarthropathy or psoriatic arthritis. Results: In all, 21.4% of the 355 families with a patient with JIA had members with type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis. Thirty‐three mothers and 23 fathers had type 1 diabetes, coeliac disease, multiple sclerosis or chronic arthritis in 15.2% (95% CI 11.6–19.4) of the families, and 23 mothers and 15 fathers had chronic arthritis in 10.7% (95% CI 7.7–14.5) of the families. When compared with available research data, the prevalences of rheumatoid arthritis, spondyloarthropathy, psoriatic arthritis, paediatric type 1 diabetes and JIA (in siblings) were increased in JIA families. Coeliac disease was as prevalent as in the population. Conclusion: Autoimmune diseases cluster in families with a child with JIA.     

Mitral and aortic insufficiency in polyarticular juvenile rheumatoid arthritis

Summary Valvar heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), the aortic valve being most commonly affected. Reported cases with symptomatic mitral involvement are rare. We describe a 13-year-old boy with seronegative, polyarticular onset of JRA in whom mitral and aortic valve insufficiency was diagnosed by clinical and laboratory investigations. Two-dimensional and continuous-wave Doppler echocardiography confirmed mild pericardial effusion with moderate mitral and mild aortic insufficiency. Cardiac assessment and echocardiogrphic follow-up are recommended in all patients with JRA.     

幼年特发性关节炎临床特征及生命质量调查

目的 了解幼年特发性关节炎患儿的急性期临床特征和远期预后,探讨与生命质量相关的临床特征及治疗方案.方法 对1997年8月至2007年8月70例幼年特发性关节炎患儿进行回顾性分析及生命质量问卷调查.结果 70例患儿中全身型占74.3%,实验室检查缺乏特异性.问卷的内部一致信度(Cronbach a 系数0.9599)和构建效度较高.76.8%患儿的生活质量评分显示良好,约17.4%留下永久性关节损害.大于5岁组生命质量明显低于小于5岁组.单用非甾体类抗类药组和非甾体类抗类药联合其他药物组的生命质量差异有统计学意义(P=0.026),发病至正规治疗的时间变量与生命质量总分的相关系数为0.329(P<0.05).握变量与"发病至正规治疗的时间、最初受累关节数目"因素显著相关.结论 大多数幼年特发性关节炎患儿预后良好.发病至正规治疗的时间、治疗情况、药物依从性、年龄是最常见的影响预后的因素,预后与最初受累关节数目、是否多系统损害也密切相关.     

儿童急性淋巴细胞白血病和幼年特发性关节炎的初步鉴别

部分急性淋巴细胞白血病患儿以骨骼肌肉表现为首发症状,这些患儿中有一部分会被误诊为幼年特发性关节炎,如何早期区分这些患儿对于及时治疗、改善预后很有意义。该文根据病史及常规的实验室检查和影像学检查,提出在疾病早期如何通过分析有关节症状患儿关节肿痛、血象及影像学特点初步鉴别儿童急性淋巴细胞白血病和幼年特发性关节炎,降低误诊率。     

血清葡萄糖-6磷酸异构酶测定在幼年特发性关节炎中的临床意义

目的了解血清葡萄糖-6磷酸异构酶(G6PI)在多关节型和少关节型幼年特发性关节炎(polyar-thritis and oligoarthritis JIA,pJIA and oJIA)及系统性红斑狼疮(SLE)患儿中的表达水平,以阐明其对pJIA和oJIA的诊断价值。方法 JIA组30例、SLE组19例和健康组37名。应用酶联免疫吸附试验(ELISA)测定三组儿童血清G6PI浓度,比较三组血清G6PI浓度、G6PI阳性率。结果 JIA组血清G6PI浓度为(0.15±0.11)μg/ml,SLE组为(0.22±0.41)μg/ml,健康对照组为(0.17±0.28)μg/ml,三组比较差异无统计学意义(P>0.05)。各组G6PI高浓度例数均为2例,各组间相比差异无统计学意义(P>0.05)。结论血清G6PI浓度测定不适用于辅助诊断关节病变为主的JIA。     

幼年特发性关节炎关节外表现诊治进展

幼年特发性关节炎(JIA)是一组原因不明,以慢性关节炎为主要特征且临床异质性较强的儿童风湿性疾病。患儿病情多数预后较好,但部分患儿可有关节外重要组织器官受累,如心血管系统损害、肺胸膜病变、肾淀粉样变性和葡萄膜炎等。如果诊治延误可致近期和/或远期重要脏器功能损害,导致病情加重或遗留严重后遗症,引起生活质量下降甚至危及生命...     

NATURAL HISTORY OF JUVENILE RHEUMATOID ARTHRITIS A Follow-up Study of a Case with Special Reference to Clinical, Electroencephalographic and Neuropathological Findings

ABSTRACT. A detailed comparison between the clinical and EEG findings is made in a case of a boy with juvenile rheumatoid arthritis (JRA) who died at 15 years, 6.5 years after the beginning of the follow-up period. In the course of the disease, seven EEG recordings were made, showing a progressive diffuse slowing and disorganization with some improvement during short remissions. In relapses, diffuse slowing was associated with grave asymmetries in the EEG which, however, fluctuated and later disappeared without accompanying clinical or neuroradiological abnormalities. An abundancy of different residual findings, however, remained in the EEG after relapses. There were spike-and-wave paroxysms in every record except at the terminal stage. A stepwise slowing and disorganization was also seen in these paroxysms as background activity. The final cause of death was an intraventricular haemorrhage. No cerebral amyloidosis was found at autopsy. In conclusion, it is suggested that JRA is also a brain disease manifested as a cerebral vasculitis.     

Physical activity assessment in children and adolescents with juvenile idiopathic arthritis compared with controls

ObjectiveWe aimed to assess physical activity (PA) in children with juvenile idiopathic arthritis (JIA) compared with healthy peers and to determine factors influencing PA level.MethodsThis was a cross-sectional study of the measured level of PA in children with JIA, compared with age- and gender-matched healthy schoolchildren. PA was estimated using a physical activity questionnaire for children and for adolescents (cPAQ/aPAQ). Disease activity was evaluated with the Juvenile Arthritis Disease Activity Score (JADAS). Functional ability was assessed with the Childhood Health Assessment Questionnaire (CHAQ).ResultsA total of 55 children with JIA and 55 healthy control schoolchildren were included. Children with JIA had significantly lower levels of PA compared with their healthy peers as assessed with the cPAQ/aPAQ (P = 0.0121). In total, 76% of the JIA group spent the day sleeping and sitting, which was significantly higher compared with the reference group (P = 0.001 and P = 0.055, respectively). Low PA level was associated with systemic JIA (P = 0.002, OR = 2.123), polyarticular JIA with positive rheumatoid factor (P = 0.001, OR = 2.014), JADAS-27 ≥ 6 (P = 0.001, OR = 2.524), patients undergoing treatment (P = 0.001, OR = 1.271), and higher CHAQ (P = 0.002, OR = 2.461).ConclusionChildren with JIA were less physically active than their healthy peers and less active than recommended for general health.     

幼年特发性关节炎白介素6、γ干扰素诱导蛋白10和白介素17的表达及意义

目的 研究幼年特发性关节炎(JIA)患儿外周血及关节液中白介素6(IL-6)、γ干扰素诱导蛋白10(IP-10)及白介素17(IL-17)的表达差异.方法 收集JIA患儿血清27例[其中全身型JIA (sJIA) 13例、多关节型JIA(pJIA) 14例]及关节液18例;疑诊sJIA患儿血清19例.另收集健康体检儿童血清28例作为对照.采用酶联免疫吸附法检测血清及关节液上清IL-6、IP-10及IL-17的浓度.结果 (1)血清细胞因子浓度:sJIA组血清IL-6浓度明显高于健康对照组[28.0(4.2 ～59.2)ng/L vs.12.3(2.1 ～ 13.8) ng/L,P＜0.05],但疑诊sJIA组与健康对照组相比无明显升高[11.8(7.7～39.2)ng/Lvs.12.3(2.1 ～13.8)ng/L,JP＞0.05].sJIA组血清IL-17浓度高于健康对照组[14.0(9.8～ 34.3)ng/L vs.9.8(7.9 ～ 16.2)ng/L,P＜0.05],pJIA组血清IL-17浓度与健康对照组相比无明显升高[14.2(9.9 ～ 16.9)ng/L vs.9.8(7.9 ～ 16.2)ng/L,P＞0.05].(2) sJIA及pJIA组关节液中IP-10的浓度均分别高于两组血清[619.7(160.9,873.1)ng/L vs.64.8(27.4 ～ 111.9) ng/L,P＜0.01;660.9(401.9,1349.8)ng/L vs.97.4(41.9 ～222.1)ng/L,P＜0.01].关节液中IL-17浓度仅pJIA组显著高于血清[22.9(17.1,45.8) ng/L vs.14.2(9.9 ～ 16.9)ng/L,P＜0.01].结论 (1)IL-6在sJIA发病中起重要作用,并且可能成为关节炎症早期的重要生物学标记.(2) sJIA发病机制中可能共同存在自身炎症反应和自身免疫反应.(3) IL-17在pJIA关节液局部高表达,而在外周血表达并不升高.(4)趋化因子IP-10在关节液和外周血中存在显著浓度梯度,可能是其发挥趋化作用,进而致sJIA关节损害的基础.     

The relationship of juvenile lumbar disc disease and Scheuermann's disease

Between 1969 and 1979 five children were found to have lumbar disc disease and were evaluated for clinically unsuspected thoracic spine abnormalities. Of these five children, two had Scheuermann's disease and one had disc space narrowing associated with Schmorl's nodes. One other had narrowed disc spaces without bony defects, and one had a normal thoracic spine. A unitary concept of childhood lumbar disc disease, Schmorl's nodes and Scheuermann's disease is suggested.