Imaging of juvenile idiopathic arthritis

Over the past decade there have been considerable changes in the classification and imaging of juvenile idiopathic arthritis (JIA). Radiology now has a considerable role in the management of JIA, the differential diagnosis, monitoring disease progression and detecting complications. The different imaging modalities available, their role and limitations are discussed in this article and the various disease features that the radiologist should be aware of are described. An approach to the imaging of the child with joint disease and in the monitoring of disease complications are also discussed.     

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.     

免疫耐受在幼年特发性关节炎发病机制中的研究进展

幼年特发性关节炎是一种免疫系统功能紊乱、调节失衡所导致的自身免疫性疾病。近年来,免疫耐受在幼年特发性关节炎发病机制中的研究日益成为热点。该文总结了近来细胞毒T细胞相关抗原-4、程序性凋亡分子-1、调节性T细胞和凋亡细胞在幼年特发性关节炎发病机制中的研究进展。     

Growth and bone mineralization in patients with juvenile idiopathic arthritis

Objective  To investigate growth, development and bone mineralization of children with juvenile idiopathic arthritis (JIA). Methods  Thirty patients between 4–17 years of age (mean 11.34 ± 3.88) resistant to therapy were studied. Enrollment began in November 1999 and continued through November 2004 and children with chronic disease were excluded. Data like height, weight, medications and acute phase reactants were obtained from medical records. On study-visit, puberty was assessed by physical examination and bone mineral density (BMD) was measured. Serum Ca, P, ALP, insulin-like growth factor-1 (IGF-1) and urinary Ca/Cr and hydroxyproline /Cr levels were measured. Results were compared with the control group that consisted of 30 cases of similar age and gender. Results  Patients with JIA had decreased height standard deviation score (SDS) and growth retardation. BMD of the cases in the study group was lower than the control group (p<0.05). Patients who were at younger age at the onset of the disease had lower BMD. Among the drugs, only steroids had a negative effect on growth. Serum IGF-1 levels of the study group were significantly lower than the control group (p<0.0001). Conclusion  Early diagnosis and suppression of disease activity is important in prevention of osteoporosis and growth retardation in children with JIA. BMD has to be measured yearly in patients for accurate diagnosis of osteoporosis. Vitamin D and Ca-rich nutrition with promotion of physical activity and controlled use of steroids may protect the children against bone loss.     

依那西普治疗非全身型幼年特发性关节炎的近期疗效及安全性观察

目的观察依那西普治疗非全身型幼年特发性关节炎的疗效和安全性。方法对30例非全身型幼年特发性关节炎患儿皮下注射依那西普0.4 mg/(kg.次),每周2次,在治疗后1周、1个月及3个月采用ACRPedi 30/50/70评分进行疗效评估。结果治疗后1周、1月及3月时ACR Pedi 30达标率分别为16.70%、90.00%、96.67%,ACR Pedi 50达标率分别为3.30%、30.00%、66.70%,ACR Pedi 70达标率分别为0、0、16.70%(5),且无重大不良事件发生。结论依那西普治疗非全身型幼年特发性关节炎具有较好的近期疗效,在病程早期使用效果更显著,不良反应小且发生率低。     

Risk of new-onset uveitis in patients with juvenile idiopathic arthritis treated with anti-TNFalpha agents

OBJECTIVE: To determine whether treatment with tumor necrosis factor alpha (TNFalpha)-blocking agents alters the incidence of new-onset uveitis in patients with juvenile idiopathic arthritis (JIA). STUDY DESIGN: Cohort study based on retrospective chart review. The charts of all 1109 patients with a diagnosis of JIA seen between January 1, 1996, and June 30, 2003, at our clinic were reviewed for diagnosis of uveitis and treatment with TNFalpha inhibitors. Cox regression analysis was performed with anti-TNFalpha treatment as a time-dependent covariate for risk of development of uveitis. RESULTS: We identified 70 patients treated with anti-TNFalpha without a prior diagnosis of uveitis. Two of these 70 patients (2.9%), both treated with etanercept, had development of new-onset uveitis during anti-TNFalpha therapy. One had juvenile psoriatic arthritis diagnosed 4.1 years before onset of uveitis. The other had extended oligoarticular JIA diagnosed 6.4 years before onset of uveitis. We found no statistically significant difference in the risk for development of uveitis between patients with or without anti-TNFalpha treatment. CONCLUSIONS: In our patients with JIA, anti-TNFalpha treatment did not alter the risk for development of new-onset uveitis. However, anti-TNFalpha therapy with etanercept did not prevent the development of uveitis in 2 patients.     

Factors Influencing the efficacy of intra-articular steroid injections in patients with juvenile idiopathic arthritis

A retrospective chart review was performed of all patients with juvenile idiopathic arthritis (JIA) followed at our clinic who had an intra-articular steroid injection between 1 January 1997 and 31 December 2001. The aim of the study was to evaluate the outcome of intra-articular steroid injections (iaS) and determine prognostic factors. During the study period, 202 iaS were performed in 60 patients, of whom 37 had oligoarticular JIA, 15 had polyarticular, rheumatoid factor-negative JIA and four each had systemic and enthesitis-related JIA. The median duration of remission was 23.1 months (range: 0–69 months). At last follow-up, 103 joints (51%) of 47 patients were still in remission after a median follow-up time of 28 months (range: 1–69 months). For the total cohort, the remission was longer for wrist and finger joints [risk ratio (RR): 0.2], with concomitant treatment with methotrexate (RR: 0.28) and for enthesitis-related arthritis (RR: 0.34). For the group of knee joints, remission was longer with concomitant treatment with methotrexate (RR: 0.37), with triamcinolone hexacetonide (RR: 0.77) and with general anaesthesia for the procedure (RR: 0.56). Mild side effects were observed in 45 iaS (22.3%), and skin atrophy occurred at the injection site in 2% of injections, but no major adverse event occurred in our cohort. In conclusion, iaS is a safe procedure with a median duration of remission of 23.1 months. The remission was longer in the joints of the upper extremity, with concomitant treatment with methotrexate and when the injection was performed under general anaesthesia.     

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...     

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。     

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目的探讨抗环瓜氨酸肽(CCP)抗体检测在幼年特发性关节炎(JIA)诊断中的意义。方法对华中科技大学同济医学院同济医院2002-06—2004-08收治的JIA患儿66例、其他风湿病患儿11例及正常儿童29名,运用酶联免疫吸附试验(ELISA)方法检测血清中抗CCP抗体,比较各组抗CCP抗体阳性率,并探讨抗CCP抗体在诊断JIA中的意义。结果66例JIA患儿中抗CCP抗体阳性率为16·7%(11/66),其中类风湿因子(RF)阳性多关节型阳性率为57·1%(4/7),RF阴性多关节型为19·0%(4/21)、少关节型为23·1%(3/13),22例全身型和3例附着点炎相关关节炎(ERA)患儿,抗CCP抗体均为阴性。其他风湿病患儿组和对照组抗CCP抗体亦为阴性。JIA组与正常对照组抗CCP阳性率比较有统计学意义(P<0·05),其中多关节型及少关节型与正常对照组比较亦有统计学意义,而其他亚型与正常对照组比较无统计学意义。结论抗CCP抗体尚不能作为JIA早期诊断的新的可靠的血清学指标。抗CCP抗体主要见于JIA多关节型和少关节型,推测抗CCP抗体对JIA分型及预后评价可能有指导意义。     

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。     

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??Abstract??Objective To investigate characteristics of clinical manifestation and therapy in children with oligoarticular and polyarticular juvenile idiopathic arthritis ??JIA??. Methods The medical records of 89 children with polyarticular or oligoarticular JIA in Children's Hospital of Chongqing Medical University from 2006 to 2011 were retrospectively reviewed. Results Totally 42 boys and 47 girls??M??F ratio??1??1.1?? were included in the study?? nearly a half ??48.31%?? were older than the age of 8 years.There were 37 cases of oligoarticular JIA??no case of extended oligoarthritis??and 52 cases of polyarticular JIA?? consisting of 10 cases of rheumatoid factor positive ??RF+?? and 42 cases of rheumatoid factor negative??RF-??. Oligoarticular and polyarticular JIA were mainly characterized with joint symptoms?? while systemic and extra-articular symptoms were rare. Oligoarthritis predominantly involved legs?? with the knee joints??28.00%?? mostly affected?? followed by the ankles ??21.33%?? and hips??17.33%??. Polyarthritis also affected the large joints at onset?? knees ??20.00%?? and ankles??18.50%???? but usually in association with small joints of the hands??18.00%?? and wrist joints??16.00%??. Chronic uvitis was recognized in 4 cases ??4.50%???? without ANA positive. Laboratory investigations were just used to help differential diagnosis. There was a high positive rate of HLA-B27??27.78%??. Sixty-one patients were treated with a combination therapy of NSAIDs and DMARDs. For the 28 refractory cases?? the treatment with tumor necrosis factor receptor-antibody fusion protein was effective without adverse reaction. Conclusion Patients suffering from polyarthritis are more than those from oligoarthritis?? which both mostly affect the school-age children. The females with polyarthritis are significantly more than males.There is a high positive rate of HLA-B27?? and low rate of antinuclear antibody and occurrence of iridocyclitis. DAS28 is a suitable criteria to evaluate clinical response in JIA. Methotrexate has been proved safe and effective for polyarthritis. Tumor necrosis factor receptor - antibody fusion protein is safe and effective to relieve the joint symptom?? which helps to improve the prognosis of JIA.     

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.     

Radiological improvement by tocilizumab in polyarticular juvenile idiopathic arthritis

Recent advances in biologic therapy have enabled reduction of the progression of destructive arthritis in rheumatoid arthritis. Once destroyed, however, the affected bones and cartilage are not fully repaired. We describe the case of an 8‐year‐old girl with anti‐citrullinated peptide antibody (ACPA)‐positive polyarticular juvenile idiopathic arthritis (p‐JIA). Destructive arthritis progressed during combination therapy with infliximab, methotrexate, mizoribine and prednisolone. Clinical remission was achieved, however, after switching the biologic agent to tocilizumab, a humanized monoclonal antibody to interleukin‐6 receptor. Both bone erosion and bone marrow edema on magnetic resonance imaging were repaired in association with restoration of joint spaces. Furthermore, there was no relapse of arthritis on weekly methotrexate alone for 2 years after discontinuation of the tocilizumab. Tocilizumab led to radiological repair of both bone and cartilage destruction and long‐term biologics‐free remission in a patient with ACPA‐positive p‐JIA, and should be considered for tumor necrosis factor inhibitor‐resistant cases.     

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????Objective To observe and evaluate the clinical effect and adverse effects of the infliximab and etanercept in treating patients with juvenile idiopathic arthritis ??JIA??.Methods There were 26 patients of JIA treated with anti-TNF therapy from June 2008 to December 2011.All patients were divided into two groups?? including those treated with in?iximab??11 patients?? and those with etanercept??15 patients??.We assessed clinical effects by DAS28??inactive disease standards and clinical remission??CRM/CR???? evaluating adverse reactions of two drugs as well.Results After a follow up of 3 to 31 months??the clinical symptoms and laboratory indexes of two groups were all improved.We have found statistical differences in CRP of infliximab at the beginning of therapy and after 3 months??in ESR of etanercept at the beginning of therapy and after 6 months??in swollen joint counts of etanercept at the beginning of therapy and after 3??12months??P??0.05????in the numbers of tender joints ??or pain with activity?? of etanercept at the beginning of therapy and after 3??6 months.Our study also showed statistically signi?cant differences in DAS28 values at the beginning of therapy and after 3??6 months in etanercept??and at the beginning of therapy and after 3 months in infliximab.There was obvious decrease of DAS28 values in etanercept compared with infliximab in the treatment of 3??6 months??but infliximab group much lower than etanercept one in 12 months.We found no signi?cant differences in short term clinical effect between the two drugs??P??0.05??.Except rushes and pain caused by injection??there were no other side effects in the two groups.Conclusion Both in?iximab and etanercept can reduce DAS28 values and improve joint function in patients with JIA.We find no signi?cant differences in the responses??remissions or adverse effects between both drugs in the short term.There are no serious adverse reactions in both groups.     

FDG‐PET in macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

We herein describe a case of systemic juvenile idiopathic arthritis (s‐JIA)‐associated macrophage activation syndrome (MAS) in which the ^18Ffluorodeoxyglucose positron emission tomography (^18FFDG‐PET) findings were characteristic. The pattern of greater ^18FFDG accumulation into the spleen compared with the liver was more remarkable in this patient compared with s‐JIA. This pattern, however, was also observed in cases of acute leukemia. In the present patient, serum interleukin (IL)‐18 was extremely elevated (255 000 pg/mL), whereas in leukemia patients it is mildly elevated (360–1480 pg/mL). ^18FFDG‐PET might be a useful indicator of s‐JIA and MAS in patients with fever of unknown origin. The pattern of ^18FFDG accumulation, however, can also be observed in acute leukemia. The combination of ^18FFDG‐PET and serum IL‐18 might be useful for the diagnosis of s‐JIA and MAS.     

Mizoribine in the treatment of rheumatoid arthritis and juvenile idiopathic arthritis

BACKGROUND: Mizoribine (MZR), isolated from culture medium of the mold, is a novel immunosuppressant developed in Japan. It has been used in patients with renal transplantation, lupus nephritis, nephrotic syndrome and rheumatoid arthritis (RA). OBJECTIVES: To review MZR in regards to mechanism of action, pharmacokinetics, efficacy and safety in the treatment of rheumatoid RA and juvenile idiopathic arthritis (JIA). RESULTS: The drug MZR inhibits both humoral and cellular immunity in RA patients. It is completely excreted in the urine within 24 h, which contributes to the safety of MZR. A series of multicenter studies indicated that MZR was effective and safe in the treatment of RA. In JIA, however, there are only a few case reports reporting its efficacy and safety. CONCLUSION: A double-blinded multicenter study is needed to establish the efficacy, safety and indication of MZR in the treatment of JIA.