幼年特发性关节炎相关葡萄膜炎42例临床特征及视力预后因素分析

目的　分析幼年特发性关节炎（JIA）相关葡萄膜炎的临床特征,探讨影响其发病及视力预后的相关危险因素。方法　收集2014年11月至2021年3月深圳市儿童医院眼科及风湿免疫科16周岁以下JIA相关葡萄膜炎患者的临床资料进行统计分析。结果　共收集JIA相关葡萄膜炎42例（单眼计71只）,其中男16例（单眼计28只）,女26例（单眼计43只）,男∶女＝1∶1.625。葡萄膜炎发病年龄4～16岁,平均发病年龄（8.8±2.6）岁。随访时间2个月至6.3年,中位随访时间2.7（1.0,4.1）年。急性病程单眼计5只（7.0%）,慢性病程单眼计66只（93.0%）,复发性病程单眼计11只（15.5%）。前葡萄膜炎单眼计60只（84.5%）,全葡萄膜炎单眼计11只（15.5%）;单侧葡萄膜炎13例（31.0%）,双侧葡萄膜炎29例（69.0%）。少关节型35例（83.3%）,RF（-）多关节型6例（14.3%）,RF（+）多关节型1例（2.4%）。血白细胞（WBC）、红细胞沉降率（ESR）、C反应蛋白（CRP）、类风湿因子（RF）检查,不同性别比较差异无统计学意义（均P＞0.05）;抗核抗体（ANA）（+）女20例,男7例,两者比较差异有统计学意义（χ2=4.747.P＜0.05）。发病年龄≤6岁、病程＞4年、初诊最佳矫正视力（BCVA）≤0.4、并发症＞2个与视力预后不佳有关（均P＜0.05）。结论　JIA相关葡萄膜炎慢性多见,常双眼发病。前葡萄膜炎是最常见的解剖类型。虹膜后粘连为最常见眼部并发症。少关节炎型JIA、ANA（+）、RF（-）的JIA患儿更易发生葡萄膜炎。视力预后与发病年龄、病程长短、初诊BCVA及出现并发症数量相关。     

幼年特发性关节炎相关葡萄膜炎诊断和治疗

幼年特发性关节炎（JIA）相关葡萄膜炎（JIA-U）是JIA的常见关节外并发症，以前葡萄膜炎多见，即虹膜睫状体炎，症状多隐匿，有致盲风险。儿童风湿科医生应加强对患者的科普宣教，并与眼专科医生紧密沟通和交流，在早期筛查、定期监测和恰当治疗三方面下功夫，维持JIA患儿的最佳视力和眼部健康。（1）筛查：根据JIA患儿不同个体的风险评估，确定眼科筛查的频度。（2）监测：建议对患有葡萄膜炎的JIA儿童进行定期的眼科监测，其间隔时间应以眼部检查结果和治疗方案为基础。（3）治疗：局部治疗，糖皮质激素应作为初始治疗，以达到控制炎症的目的。全身治疗、甲氨蝶呤和单克隆抗体类肿瘤坏死因子拮抗剂被推荐用于葡萄膜炎的全身治疗。局部治疗失败或依赖者，建议及时添加传统、生物制剂改善风湿病情的药物（DMARD），使有视力丧失风险的葡萄膜炎患儿尽快得到病情控制，避免失明。     

结节性脂膜炎并葡萄膜炎误诊1例

结节性脂膜炎又称Weber-Christian病,临床特征为反复发热、皮下结节及非游走性关节炎,并葡萄膜炎少见.本院2008年7月收治1例结节性脂膜炎并葡萄膜炎患儿.     

MTX治疗幼年类风湿性关节炎患儿的难治性慢性葡萄膜炎

幼年类风湿性关节炎 ( JRA)最易引起儿童慢性葡萄膜炎 ,皮质类固醇局部治疗可控制大部分葡萄膜炎但并非对所有病例有效。报道用小剂量氨甲蝶呤( MTX)治疗 7例 JRA相关性慢性活动性葡萄膜炎的经验。病人和方法　 1994年 1月～ 1996年 12月对华盛顿市某医院 JRA相关性葡萄膜炎患儿应用 MTX治疗 ,7例患儿均有一年以上慢性活动性葡萄膜炎病史 ,用皮质类固醇未能完全控制且有进行性类固醇相关性白内障和青光眼。先给予皮质类固醇滴眼 ,无效时再全身应用或眼周注射类固醇 ,MTX按 0 .5～ 1mg/ kg每周一次皮下注射 ,每月测定血肌肝、全血细…     

218例幼年特发性关节炎血液系统的临床表现

幼年特发性关节炎(juvenileidiopathicarthritis,JIA)是儿童常见的结缔组织病之一,常合并慢性病贫血(anemiaofchronicdisease,ACD)或称炎症性贫血(inflamatoryanemia)。1994年6月～2004年8月,我科收住了218例JIA,乃回顾分析本病在血液系统的异常临床表现及实验室检查,以加深JIA     

生物制剂治疗幼年特发性关节炎新策略

幼年特发性关节炎(juvenile idiopathic arthritis,JIA)是儿童时期最常见的风湿性疾病之一,预后较差.生物制剂改变了JIA的传统治疗模式,使JIA患儿的病情明显改善.JIA是一种异质性疾病,采用生物制剂治疗前要评估其病情,根据其不同的临床表现选择相应的治疗方案.治疗过程中也要评估临床疗效及不良反应,平衡治疗的风险和益处.该文重点介绍生物制剂在JIA中的应用现状,并根据2011年美国风湿病学会发布的治疗JIA的循证建议对生物制剂的选择进行阐述.     

幼年类风湿性关节炎并葡萄膜炎39例

目的探讨幼年类风湿性关节炎(JRA)并葡萄膜炎的临床特征、诊断及治疗。方法对1995年3月~2004年4月我科就诊的39例JRA并葡萄膜炎患儿进行眼科裂隙灯、B超、眼底、ESR、类风湿因子(RF)、ASO及胸部X线检查,并分析患儿预后及并发症情况。结果20例经治疗眼病症状消失,10例有不同程度虹膜后黏连,3眼并白内障,3眼继发青光眼,2眼仅存光感,1眼眼球萎缩。病程3个月左右。结论JRA并葡萄膜炎多为慢性复发性前葡萄膜炎,病程隐匿,其典型特点是均有关节炎病史、发病年龄在16岁以下的前葡萄膜炎。常用治疗药物为糖皮质激素、睫状肌麻痹剂(如阿托品)、免疫抑制剂。儿科医师应重视JPA患儿眼科情况,眼科医生应熟悉眼与全身病的关系,以便其能在第一时间得到合理治疗,减少致盲率。     

降钙素原在幼年特发性关节炎诊断中的临床意义

目的检测幼年特发性关节炎(JIA)血清降钙素原(PCT)改变,探讨PCT在JIA诊断中的临床意义。方法检测2011年1月至2012年12月湖北省武汉市妇女儿童医疗保健中心风湿免疫科150例JIA患儿血清PCT和C反应蛋白(CRP)值,比较PCT和CRP对诊断JIA细菌感染的敏感度、特异度、阳性预测值、阴性预测值。同时,检测PCT在JIA各种临床类型的表达情况。结果 JIA细菌感染组血清PCT与CRP值,均明显高于JIA病毒感染组、JIA活动不伴感染组及对照组,差异均有统计学意义(P均0.05)。以PCT≥0.5μg/L及CRP≥8 mg/L为诊断细菌感染的阳性阈值,两指标敏感度分别为76.2%、85.7%,特异度分别为87.6%、51.9%,阳性预测值分别为50.0%、21.2%,阴性预测值分别为95.8%、95.4%,阳性似然比6.14、1.65,阴性似然比0.27、0.30。ROC曲线下面积:PCT为0.928,优于CRP(0.714),差异有统计学意义(u=2.19,P0.05)。98.99%(98/99)JIA活动不伴感染组PCT值0.5μg/L,中位数为0.2μg/L。66.7%(66/99)JIA活动不伴感染组PCT值0.1μg/L。结论血清PCT值对JIA并发细菌感染具有重要鉴别意义,其预测感染价值优于CRP。推荐PCT值0.5μg/L作为诊断JIA合并感染临界值。     

幼年特发性关节炎细胞因子基因多态性研究进展

幼年特发性关节炎(JIA)是关节滑膜的慢性炎症,而细胞因子是炎症反应的重要介质和调节剂,细胞因子的作用在JIA的发病机制中十分重要.细胞因子的基因多态性可以改变基因的表达,直接影响到人类对JIA的易感性.通过细胞因子基因多态性与.JIA易感性的相关研究,有利于早期发现JIA高危易感人群,早期干预治疗,从而减少JIA后遗症的发生.该文就近年来国外研究较多的几种细胞因子基因多态性与JIA的关系作一综述.     

幼年特发性关节炎免疫致病机制研究新进展

幼年特发性关节炎(JIA)是一组以慢性关节炎为主伴有全身多系统受累的自身免疫性疾病,致病机制相当复杂,以不明原因的慢性关节炎为特征.JIA定义为16岁以下不明原因的关节肿胀并持续6周以上的关节炎,起病方式各异,临床表现多样,一些病例呈现一定的遗传背景.国际风湿病学联盟2001年统一命名,将其分为7种亚型.JIA的致病机制至今不明,各类亚型的异质性可能与遗传以及环境因素相关.JIA遗传背景复杂,研究发现人类白细胞抗原、细胞因子与T细胞调节相关的等位基因与JIA发病有关.尽管JIA-直被认为是由T细胞异常识别自身抗原而引起的自身免疫病,但近年研究显示,JIA更可能是崮有免疫与获得性免疫交互作用的结果.除了遗传因素外,近几年体液免疫与细胞免疫方面的研究也有一定的进展.     

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.     

幼年特发性关节炎37例

目的探讨幼年特发性关节炎(JIA)的临床特点及治疗方法。方法按照国际风湿病学联盟(ILAR)新的分类标准对JIA患儿进行分型,总结37例JIA患儿的临床表现、实验室和影像学检查结果及药物治疗及其转归。结果全身型22例(59.46%),少关节型7例(18.92%),多关节型5例(13.51%),其他关节炎型3例(8.10%)。全身型以非类固醇性抗炎药(NSAIDs) 缓解病情抗风湿药物(DMARDs) 激素治疗为主,其中8例加用细胞毒药物(CTX);少关节型、多关节型及其他关节炎型以NSAIDs DMARDs 小剂量激素治疗,基本能够控制病情,无1例发生关节功能障碍。结论JIA以全身型最多见,其次为少关节型;JIA治疗应提倡早期联合治疗,以尽快控制炎症,改善病情,防止残疾发生。     

Die rheumatische Uveitis im Kindesalter

Uveitis in childhood is frequently associated with underlying rheumatic diseases, especially with juvenile idiopathic arthritis (JIA). Chronic iridocyclitis (uveitis anterior), which is observed together with JIA-oligoarthritis, JIA-rheumatic factor-negative polyarthritis, JIA-psoriatic arthritis or sarcoidosis, is often followed by irreversible, sight-threatening iridocyclitis complications. Acute iridocyclitis, which is typical for JIA-enthesitis-associated arthritis, for juvenile spondarthritis or juvenile reactive arthritis including Reiter's syndrome, on the other hand, has a more favourable prognosis. Most important for a favourable prognosis of such cases of uveitis are the early diagnosis, that is to say a diagnosis before irreversible eye complications have appeared, and the early onset of a competent treatment. This is made possible by the use of prophylactic slit-lamp examinations which, depending on the risk of developing uveitis complications, are to be performed routinely once every 4 weeks to once every 6 months. It is the paediatrician's responsibility to identify the children at risk, to initiate the mandatory diagnostic measures and to monitor the follow-up.     

Uveitis and anti nuclear antibody positivity in children with juvenile idiopathic arthritis

This study was conducted to determine the frequency of antinuclear antibodies (ANA) positivity and uveitis in our newly diagnosed juvenile idiopathic arthritis (JIA) patients classified according to International League Against Rheumatology (ILAR) classification criteria. Ninety-two girls and 106 boys, totally 198 children were enrolled in the study. of them 36 (18.2 percent) were found to be ANA positive. Chronic anterior uveitis was detected in 20 (10.1 percent) patients. ANA positivity was determined in 4 of the systemic JIA patients, in whom no uveitis had been detected. Twenty-five of 37 patients with oligoarticular JIA were ANA positive, in 10 of them uveitis was also diagnosed. ANA were positive in 3 of 34 patients with RF positive polyarticulat JIA, only one patient had positive ANA, and another one had uveitis. Nine patients were extended JIA and in none of them, ANA positivity or uveitis were present. Of 43 patients classified as enthesitis related arthritis (ERA), uveitis was diagnosed in 6 and there was no evidence of ANA positivity, but one had uveitis. We conclude that the incidence of ANA positivity and uveitis is low in Turkish children with JIA.     

幼年特发性关节炎228例

目的探讨幼年特发性关节炎(JIA)的临床特点、分类及治疗措施。方法总结1994年1月-2005年10月本院儿科收治的228例JIA患儿起病特点、临床症状、体征、家族史及辅助检查资料,以2001年国际风湿病学联盟儿科专家组制订的JIA标准进行分类,并对JIA患儿的临床表现、实验室检查、治疗和转归进行回顾性分析。选取同期本院健康儿童48例作对照。结果本组JIA患儿男151例,女77例。全身型JIA93例,少关节型JIA50例,多关节型JIA51例,与附着点炎性反应相关的关节炎(ERA)34例;临床表现极不典型,以发热、关节病变、皮疹、疲乏、纳差等多见;99例患儿并轻或中度贫血;免疫学检测发现JIA患儿存在着明显的细胞免疫和体液免疫紊乱;70例患儿有心脏损害,主要表现为心肌酶升高、心律失常、心脏结构或心瓣膜病变。结论掌握JIA的临床表现及辅助检查结果有助于明确诊断。全身型JIA最为多见,其次为多关节型JIA。JIA临床表现多样,治疗应依据不同临床分型予以不同治疗方案,则能尽量改善患儿病情,提高患儿学习、生活质量。     

Uveitis bei juveniler idiopathischer Arthritis

Uveitis adds to the problems in children and adolescents with juvenile idiopathic arthritis (JIA), influencing the prognosis substantially and in ways that are quite independent of the arthritis itself. The Working Group for Children and Adolescents with Rheumatic Diseases in Germany (AGKJR)—and especially the branch group in North Rhine-Westphalia—and the German Uveitis Centers have initiated the formulation of an evidence-based strategy for the management of uveitis in JIA. This gives recommendations on the content and frequency of ophthalmological examinations and also a treatment concept. The aim is to improve the clinical management of these patients and to create a platform for further scientific approaches with uniform strategies.     

Juvenile idiopathic arthritis profile in Turkish children

Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders. Publications from different countries point to differences in the disease manifestation of JIA among different populations. The aim of the present paper was to evaluate the clinical and laboratory features of JIA in Turkish children. Methods: A total of 196 JIA patients who fulfilled International League of Associations for Rheumatology (ILAR) diagnostic criteria were included in this retrospective study. The data collected were age, gender, age at disease onset and at diagnosis, and follow‐up duration. Antinuclear antibody (ANA), rheumatoid factor (RF), and human leukocyte antigen B‐27 were evaluated for each patient. Results: There were 102 boys and 94 girls with a mean duration of disease of 4.1 years. The mean age at the first visit was 8.8 years, and the mean age at onset of disease was 6.8 years (range, 8 months–15 years). Polyarticular JIA was the most frequent onset type (37.2%). Other subtypes included oligoarthritis (34.2%), systemic arthritis (15.3%), psoriatic arthritis (1%), enthesitis‐related arthritis (9.7%), and other arthritis (2.2%). ANA was positive in 28 patients (14.2%). Chronic uveitis occurred in two patients with oligoarthritis; and two patients with enthesitis‐related arthritis had acute uveitis. Three patients (1.4%) developed amyloidosis. Conclusion: Compared to reports from Western countries, remarkably different features of JIA were found in Turkish children, which included higher frequency of polyarticular JIA, higher prevalence among boys, lower rate of ANA positivity and uveitis. Further studies are required to understand how genetic and environmental differences affect JIA expression.     

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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.