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。     

幼年特发性关节炎肺胸膜病变的相关因素分析

目的 探讨幼年特发性关节炎（JIA）患儿肺胸膜病变（PLD）的高危因素,为临床判断病情、及时处理提供依据。方法 回顾分析360 例JIA 患儿的临床资料,所有患儿均行胸部X 线影像学检查,将患儿分为合并PLD 组和无PLD 组,分析JIA 伴肺胸膜病变（JIA-PLD）患儿的临床、影像学及相关实验室指标。结果 360 例JIA 患儿中,JIA-PLD 共43 例（11.9%）,其中有呼吸系统症状者9 例（21%）。胸部影像学异常主要表现为间质性肺炎（53.5%）,其次为胸膜炎和/或胸腔积液（38.1%）。43 例JIA-PLD 中胸片正常而胸部CT 异常者4 例（9.3%）。P结论 JIA-PLD 多发生于全身型JIA;PLD 好发于年龄<3 岁或≥ 12 岁者;伴有贫血、外周血WBC 及免疫球蛋白G 升高、类风湿因子或抗核抗体阳性者PLD 发生率高。PLD 影像学多表现为间质性肺炎,但由于患儿多缺乏呼吸系统表现,建议常规高分辨率胸部CT 检查以便临床早期发现、及时处理。     

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.     

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.     

幼年特发性关节炎患儿肾脏受累的观察

目的 观察幼年特发性关节炎(JIA)患儿肾脏受累的特点以及与临床分型、病程、用药之间的关系.方法 回顾性分析60例JIA患儿的临床表现,并进行血沉、C反应蛋白、类风湿因子、血生化、免疫球蛋白、补体、抗核抗体、尿常规、尿系列蛋白等辅助检查.其中2例患儿接受肾穿刺活检.结果 17例(28.3%)患儿出现尿常规异常,依次为全身型7例(7/18,占38.9%)、多关节型6例(6/22,占27.3%)、少关节型4例(4/20,占20.0%).表现为孤立性血尿和(或)蛋白尿,其中1例全身型患儿合并肾小管损害,另有5例(8.3%)伴血清尿素氮增高.肾脏受累的JIA患儿中病程<1个月者12例(70.6%),初发未用药和短期(<2周)口服非甾体抗炎药者10例(58.8%).结论 血尿、蛋白尿是JIA患儿肾脏累及最常见的临床表现,以全身型较多见,其中原发疾病所致肾损害可能性较大.随访尿常规和肾功能,积极治疗原发病,将有助于减少肾脏病变的发生.     

幼年特发性关节炎合并葡萄膜炎15例病例系列报告

背景 葡萄膜炎为幼年特发性关节炎(JIA)最常见的关节外表现,治疗不及时可导致严重并发症,有致盲风险.目的 探讨JIA合并葡萄膜炎的临床特征.设计病例系列报告.方法 纳入首都儿科研究所附属儿童医院风湿免疫科2018年3月至2020年5月收治的JIA合并葡萄膜炎患儿,从病历中截取人口学资料、实验室检查、JIA分型、眼部表...     

不同亚型幼年特发性关节炎患儿血脂水平变化研究

目的分析不同亚型幼年特发性关节炎（JIA）患儿活动期及缓解期血脂水平的变化，初步探讨JIA患儿远期发生动脉粥样硬化的风险。方法将128例初诊为JIA活动期患儿根据亚型分为少关节型48例，多关节型38例，全身型22例，附着点型20例，其中38例多关节型JIA患儿又根据类风湿因子（RF）是否阳性分为RF阳性多关节型15例，RF阴性多关节型23例；同期另随机选取45例行健康体检儿童作为健康对照组。检测各组血清总胆固醇（TC）、甘油三酯（TG）、低密度脂蛋白胆固醇（LDL-C）、高密度脂蛋白胆固醇（HDL-C）等水平进行比较分析。对87例治疗后处于缓解期的患儿进行血脂指标的复查，并与活动期各血脂指标水平进行比较分析。结果在JIA活动期，全身型、RF阳性多关节型组与健康对照组比较，HDL-C水平明显下降，TG水平明显升高（P < 0.05），而TC、LDL-C水平差异无统计学意义（P > 0.05）；其他亚型各血脂指标水平与健康对照组比较差异均无统计学意义（P > 0.05）。RF阳性多关节型JIA患儿缓解期与活动期比较，血浆HDL-C明显升高（P < 0.05）；其他亚型JIA患儿缓解期与活动期比较，血浆各血脂指标水平差异均无统计学意义（P > 0.05）。结论全身型、RF阳性多关节型JIA活动期存在血脂代谢紊乱，RF阳性多关节型JIA缓解期血脂紊乱有所改善，其远期发生动脉粥样硬化的风险是否增大尚需进一步观察。     

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.     

Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis

Background  Macrophage activation syndrome (MAS) is a severe, potentially life-threatening condition induced by chronic rheumatic diseases, especially systemic-onset juvenile idiopathic arthritis (SoJIA) in childhood. This study aimed to analyze the clinical and laboratory characteristics of systemic-onset juvenile idiopathic arthritis (SoJIA) with macrophage activation syndrome (MAS) in 13 patients. Methods  Clinical and laboratory data of 13 SoJIA patients with MAS treated in our hospital from January 2003 to October 2007 were analyzed. Results  In the 13 patients, 9 were boys and 4 girls aged from 5 months to 12 years. Clinical manifestations were of no typical characteristics including persistent fever, anemia, arthritis, hepatosplenomegaly, lymph-adenopathy, dysfunction of the liver, abnormal fat metabolism, and hemophagocytic cells in the bone marrow. Two patients experienced acute respiratory distress syndrome, two had mutiorgan failure, and three died. The perforin A91V (NCBI:SNP rs35947132) gene in 6 patients was normal. Glucocorticoid and immunoimpressive therapy were effective in all patients and plasmapheresis used in one severe patient was also effective. Conclusions  MAS is a serious complication of JIA, especially systemic-onset juvenile idiopathic arthritis. It is essentially important to recognize and treat MAS earlier in order to lower the mortality.     

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...     

Quantitative MR characterization of disease activity in the knee in children with juvenile idiopathic arthritis: a longitudinal pilot study

Background The development of a quantifiable and noninvasive method of monitoring disease activity and response to therapy is vital for arthritis management. Objective The purpose of this study was to investigate the utility of quantitative dynamic contrast-enhanced MRI (DCE-MRI) based on pharmacokinetic (PK) modeling to evaluate disease activity in the knee and correlate the results with the clinical assessment in children with juvenile idiopathic arthritis (JIA). Materials and methods A group of 17 children with JIA underwent longitudinal clinical and laboratory assessment and DCE-MRI of the knee at enrollment, 3 months, and 12 months. A PK model was employed using MRI signal enhancement data to give three parameters, K^{trans ′} (min⁻¹), k_ep (min⁻¹), and V_p ^′ and to calculate synovial volume. Results The PK parameters, synovial volumes, and clinical and laboratory assessments in most children were significantly decreased (P < 0.05) at 12 months when compared to the enrollment values. There was excellent correlation between the PK and synovial volume and the clinical and laboratory assessments. Differences in MR and clinical parameter values in individual subjects illustrate persistent synovitis when in clinical remission. Conclusion A decrease in PK parameter values obtained from DCE-MRI in children with JIA likely reflects diminution of disease activity. This technique may be used as an objective follow-up measure of therapeutic efficacy in patients with JIA. MR imaging can detect persistent synovitis in patients considered to be in clinical remission.     

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。     

Benefit of fluoroscopically guided intraarticular,long-acting corticosteroid injection for subtalar arthritis in juvenile idiopathic arthritis

Background Children with arthritis may endure a lifetime of disfigurement, dysfunction, and pain if acute inflammation progresses to chronic changes in the joint cartilage and underlying bone. Intraarticular steroids have become an integral component of treatment, but at times are difficult to deliver to joints, such as the subtalar joint, that have complex anatomies. Objective We describe our technique and outcomes using fluoroscopically guided intraarticular subtalar steroid injection in patients with active symptoms of juvenile idiopathic arthritis (JIA). Materials and methods Fluoroscopically guided subtalar joint injections were performed in 38 children (mean age 6.7 years). Medical records were reviewed retrospectively and improvement was evaluated clinically by the degree of foot movement in eversion and inversion. Results Subtalar joint injection was technically successful in 100% of the JIA patients with improvement in physical symptoms in 34/38 (89%). Of the 38 children, 32 were followed up within 13 weeks of the initial injection and, therefore, satisfied the eligibility criteria for resolution of arthritis. Of these 32 children, 14 showed clinical resolution (44%). The mean duration of improvement was 1.2 ± 0.9 years. Children with a longer interval (>1 year) from diagnosis to treatment had significantly less resolution (P = 0.04). Local subcutaneous atrophy or hypopigmentation were observed in 53% of the children after steroid injection (20/38). These minor complications were associated with a greater volume of steroid injected into the site per child (P = 0.02). Conclusion Fluoroscopically guided subtalar joint injection is an effective treatment for subtalar arthropathy. Prompt referral for intraarticular steroid treatment in the acute phase improves response. Skin changes often occur at the injection site, and specific precautions should be employed to reduce this risk. Prospective study is indicated to determine the most effective treatment strategy to prevent long-term pain and disability.     

新发活动期全身型幼年特发性关节炎血清细胞因子水平分析

目的 分析新发活动期全身型幼年特发性关节炎(SJIA)患儿血清细胞因子水平,探讨细胞因子在其发生发展中的作用.方法 采用流式细胞术检测2010年1月至2013年12月新发活动期74例SJIA患儿血清白介素(IL)-2、-4、-6、-10、肿瘤坏死因子(TNF)和γ-干扰素(IFN-γ)水平,202例健康体检儿童为对照组;并同时检测患儿白细胞、中性粒细胞、血红蛋白、血小板、超敏C反应蛋白和血沉等常规实验室指标.结果 74例SJIA患儿的白细胞、中性粒细胞比例、超敏C反应蛋白和血沉等均明显高于正常范围,血小板在正常范围,血红蛋白水平低于正常范围.与健康对照相比,SJIA组患儿血清IL-6明显升高(PPP>0.05).SJIA患儿IL-6水平明显升高,血红蛋白明显降低,两者呈负相关(r=-0.244,P结论 SJIA患儿血清IL-6水平明显升高,且与贫血呈负相关.     

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.     

幼年特发性关节炎患儿丹参注射液治疗前后血D-二聚体的变化

目的 观察幼年特发性关节炎(JIA)患儿病情活动时D-二聚体的变化及丹参注射液辅助治疗的疗效.方法 将47例JIA患儿随机分配为治疗组(32例)和对照组(15例),两组按JIA常规治疗,治疗组加用丹参注射液,两组患儿在治疗前和治疗后14 d测定D-二聚体、血沉(ESR)、C反应蛋白(CRP),结合临床疗效评估结果进行比较.结果 JIA患儿病情活动时D-二聚体增高,与治疗前比较,治疗后D-二聚体、ESR、CRP同步下降,差异有统计学意义(P<0.01;P<0.05);治疗组D-二聚体降低程度较对照组明显,差异有统计学意义(P<0.05);治疗组临床有效率(59.4%)与对照组(26.7%)比较,差异有统计学意义(X2=4.372,P<0.05).结论 D-二聚体可作为JIA病情活动指标之一;丹参注射液可辅助治疗JIA,改善微循环和机体状态.