Leflunomide in systemic onset juvenile idiopathic arthritis

Methotrexate, the mainstay of treatment in Juvenile idiopathic arthritis, might not be effective in a few patients of polyarticular and systemic onset juvenile idiopathic arthritis. Use of biologicals like TNF-α blockers, the next line of preferred drugs is constrained by the high cost. We successfully used leflunomide in four patients.     

Aerobic capacity and disease activity in children, adolescents and young adults with juvenile idiopathic arthritis (JIA)

ABSTRACT: OBJECTIVE: As patients with juvenile idiopathic arthritis (JIA) progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovascular morbidity and mortality in late adulthood. The objectives of the current study are to examine the aerobic capacity and its relation to parameters of disease activity in children, adolescents and young adults with JIA. METHODS: Sixty-three patients with JIA (aged 10-27 years) were cross sectional studied regarding their aerobic capacity and correlations were made to demographic, disease-related variables, and medication utilization. in a cross-sectional study group of 63 patients of all subtypes. Patients were divided in three age groups, 10-13 years; 14-17 years and 18-27 years. RESULTS: Reduced aerobic capacity is found in clinical remission as well as active disease in all subtypes and all age groups. Aerobic capacity is more impaired in active disease shown by DAS 28, JADAS 27, ESR and serum thrombocyte counts. Lower haemoglobin has a negative impact. Long-term used medication including methotrexate and corticosteroids didn't influence outcome. There is no association with current sports participation. CONCLUSION: Reduced aerobic capacity is present in adolescents and young adults with JIA, both in active disease and in patients with remission. Measures of aerobic capacity may serve as important outcome measure in JIA.     

全身型幼年特发性关节炎发病机制研究进展

幼年特发性关节炎(JIA)是一组16岁以前起病,原因不明,以慢性关节炎为主要特征,可伴有其他组织、器官损害的慢性全身性疾病,并除外其他疾病所致关节炎.与其他类型JIA相比,全身型JIA(sJIA)常伴有特殊的临床表型,如弛张高热、皮疹、浆膜腔积液、肝脾淋巴结肿大及多器官损害,少数甚至伴发巨噬细胞活化综合征(MAS).此...     

Serum thrombomodulin in systemic lupus erythematosus and juvenile idiopathic arthritis

Thrombomodulin is a thrombin receptor on the vascular endothelial cell surface which is likely released upon endothelial cell damage. Serum soluble thrombomodulin (sTM) was assessed and investigated as a parameter of disease activity in children and adolescents with systemic lupus erythematosus (SLE) and juvenile idiopathic arthritis (JIA). Patients included in this study were regularly attending the Allergy and Immunology Clinic, Children's Hospital, Ain Shams University. They were 38 (76%) females and 12 (24%) males, their ages ranged between 5 and 18 years with a mean of 14.3 +/- 4.84 years and median of 13 years. They were divided into two groups: SLE group which included 20 patients and JIA group which included 30 patients; and the control group which included 30 healthy age and sex-matched individuals for comparison. Disease activity in SLE patients was evaluated by systemic lupus erythematosus disease activity index (SLEDAI) score, while in JIA patients disease activity was determined by number of joints with active arthritis, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Serum levels of sTM were determined by enzyme-linked immunosorbent (ELISA) assay. Serum levels of sTM were significantly higher in SLE and JIA patients in comparison with the control group; there was no significant difference between SLE and JIA patients. In SLE patients, a highly significant correlation was found between sTM and SLEDAI score (r = 0.99, p < 0.001). In JIA patients, a highly significant correlation was found between sTM and number of joints with active arthritis as well as ESR (r = 0.85, p < 0.001; r = 0.93, p < 0.001, respectively). Levels of sTM were significantly higher in CRP-positive than CRP-negative JIA patients. Serum sTM is a useful serologic marker of disease activity in SLE and JIA. It may prove to be a potential indicator for early and more aggressive treatment. Furthermore, sTM may prove to be an important marker for vasculitis in general.     

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。     

托珠单抗治疗难治性全身型幼年特发性关节炎的中长程疗效

目的总结分析托珠单抗治疗难治性全身型幼年特发性关节炎的治疗经验,探索经济有效治疗方法。方法回顾分析2014年至2016年6例使用托珠单抗的难治性全身型幼年特发性关节炎患儿临床资料,从托珠单抗疗程、疗效、激素减量情况、不良反应及生长等方面进行描述和分析。结果 6例患儿平均年龄6岁,男女比例为1:1,病程16至63个月;均在激素及传统抗风湿病药物治疗基础上联合使用其他免疫抑制剂或生物制剂。托珠单抗治疗时间7~26个月,中位数为9.5个月。6例患儿均在托珠单抗治疗后不同时间内达到临床缓解。3例患儿在诱导缓解后用药间隔由2周渐延长,最长至4周,未出现疾病活动。除1例患儿外,其余5例平均可在托珠单抗治疗开始后5.8个月内减停激素,激素减停后生长改善。6例患儿在托珠单抗治疗期间均未出现严重不良反应。结论托珠单抗治疗难治性全身型幼年特发性关节炎安全有效,可在短期内减停激素,改善生长;可尝试在诱导缓解后延长用药间隔。     

幼年特发性关节炎全身型早期诊断标准探讨

目的通过回顾性分析与比较幼年特发性关节炎全身型(systemic onset juvenile idiopathic arthritis,SOJIA)与其他疾病引起的发热待查患儿的临床特点及实验室检查结果,来获得鉴别诊断的临界值,以提高早期诊断SOJIA的准确性。方法收集不明原因发热患儿共124例,记录患儿持续发热达2~4周时的临床特点和实验室检查结果,并随访1年以上。对资料完整的病例采用SPSS16.0软件进行单因素分析,对有统计学意义的单因素进行受试者工作特征曲线(ROC)分析,然后对有意义的临床特点和实验室检查结果进行联合分析。结果诊断明确且资料完整者共96例,其中33例为SOJIA,19例为其他自身免疫性疾病,25例为血液/肿瘤疾病,19例为感染性疾病。当血清铁蛋白(SF)≥545.75 ng/ml时,可以将SOJIA与其他自身免疫性疾病区分的灵敏度为97.0%,特异度为100%,若以出现皮疹或关节肿/痛加上血小板计数≥217×109/L或C3≥1.275 g/L作为标准,其区分SOJIA和血液肿瘤疾病的灵敏度为100%,特异度为96%。若以SF≥441.7ng/ml加上出现皮疹或关节肿/痛,那么其区分SOJIA和感染性疾病的灵敏度和特异度均可达到100%。结论结合发热待查患儿的临床特点及实验室检查结果可提高早期诊断SOJIA的准确性。     

幼年特发性关节炎全身型并发巨噬细胞活化综合征24例临床分析

目的总结巨噬细胞活化综合征的临床特征、可能的诊断指标、治疗方法及转归,提高对本病的认识。方法回顾性分析2003年3月至2006年2月,我院收治的24例幼年特发性关节炎全身型（SOJIA）合并巨噬细胞活化综合征（MAS）患者的临床资料,分析其临床表现、早期特征、诊断标准、可能的诱因、治疗和转归。结果24例患者,男21例,女3例,平均年龄7岁。临床表现全部患者均有高热、肝脾或（和）淋巴结进行性增大、血液系统受累,12例有中枢神经系统功能障碍,9例有易出血现象,6例有呼吸系统受累（ARDS）,6例有消化系统表现,5例心脏受累。实验室检查均有血细胞减低、血清肝酶增高、乳酸脱氢酶增高、红细胞沉降率降低、高铁蛋白血症、钠离子减低、白蛋白减低及凝血功能异常,骨髓中发现吞噬血细胞,20例患者有甘油三酯增高。治疗应用甲泼尼龙加环孢素A可以达到较好的疗效。结论MAS是SOJIA的一个致死性并发症,可以造成全身各脏器的功能衰竭。提高认识、早期诊断并积极治疗是减少死亡率的关键。治疗给予甲泼尼龙冲击及环孢素A治疗往往能得到较好的疗效。     

幼年特发性关节炎全身型并发巨噬细胞活化综合征一例

巨噬细胞活化综合征（MAS）为儿童慢性风湿性疾病少见但凶险的合并症,近来国外有所报道,国内病例尚少报道,我科2003年12月收治1例幼年特发性关节炎（JIA）全身型合并MAS,现报告如下。     

幼年特发性关节炎并发巨噬细胞活化综合征1例报告

患儿,男,11岁。因“反复发热4月余,发现白细胞减少1d”于2006年6月入院。入院前外院行骨穿等检查后诊断为“幼年类风湿性关节炎”,并给予糖皮质激素和环磷酰胺（CTX）治疗,患儿体温正常,但出现外周血白细胞下降,故至我院就诊。     

非甾体类消炎药联合甲氨蝶呤治疗全身型幼年特发性关节炎疗效观察

目的探讨非甾体类消炎药(NSAIDs)联合甲氨蝶呤(MTX)治疗全身型幼年特发性关节炎(SO-JIA)的疗效。方法分析比较32例SO-JIA患儿以NSAIDs联合MTX治疗后临床症状的变化,关节炎病情改善评估参照美国风湿病学会推荐的类风湿性关节炎改善标准。结果32例患儿治疗后,体温恢复正常27例,退热有效率为84.4%(27/32例),5例加用糖皮质激素后热退;31例关节炎症状改善,有效率为96.9%(31/32例),1例加用来氟米特后关节炎症状缓解。停药观察3例,复发1例。结论SO-JIA诊断确立后应尽早予NSAIDs联合MTX治疗,NSAIDs能较好地退热及减轻关节症状,MTX可有效持续改善关节炎症。糖皮质激素不作为首选退热用药。     

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.