风湿病及结缔组织病

071769幼年特发性关节炎的诊治进展/幺远…∥中国误诊学杂志.-2007,7(3).-448~450071770与附着点炎症相关幼年特发性关节炎临床分析/周纬…∥中国当代儿科杂志.-2007,9(1).-77~7825例有前驱感染史9例(36%),患儿一级亲属中患有强直性脊柱炎8例(32%)。NSAIDs联合SASP治疗,23例患者关节症状改善,2例因持续关节肿胀、血沉升高加MTX,MTX服用时间分别为15周和16周,3例并发虹膜睫状体炎患儿使用糖皮质激素。参7(林榕)071771幼年特发性关节炎临床特征与治疗/王叶红…∥中国现代医学杂志.-2007,17(3).-379~381对29例幼年特发性关节炎(JIA)患…     

幼年特发性关节炎28例诊断治疗分析

目的　探讨幼年特发性关节炎 (JIA)临床特点及治疗。方法　总结 2 8例JIA患儿的临床表现、实验室和影像学检查结果 ,以国际风湿病学联盟 (ILAR)儿科专家组新的分类标准讨论稿 (加拿大埃得蒙顿 ,2 0 0 1)进行分析。结果　全身型JIA 7例 ,用非甾体类消炎药 (NSAIDs)治疗 ,3例有效 ,2例部分效应 ,2例无效 ;部分效应和无效者加用糖皮质激素治疗 ,其中 1例联合甲氨蝶呤 (MTX)治疗。少关节型JIA 2例 ,以NSAIDs治疗。多关节型JIA类风湿因子 (- ) 4例 ,多关节型JIA类风湿因子 (+) 2例 ,用NSAIDs联合MTX治疗 ,以MTX维持治疗。与附着点炎症相关性关节炎 13例 ,以NSAIDs联合柳氮磺吡啶 (SASP)治疗 ,其中 2例加用MTX ,2例并发虹膜睫状体炎加用糖皮质激素 ,以SASP维持治疗。结论　JIA中 ,与附着点炎症相关性关节炎最为多见 ,其次为全身型JIA ;治疗应依据临床分型 ,联合用药并注意个体化 ;MTX对各型JIA均有较好疗效。     

全身型幼年特发性关节炎并巨噬细胞活化综合征9例

目的探讨幼年特发性关节炎(SO-JIA)并巨噬细胞活化综合征(MAS)患儿的临床特点,为儿童SO-JIA并MAS的诊疗提供临床依据。方法回顾性分析2010年5月-2011年10月于上海交通大学医学院附属仁济医院儿科住院的9例SO-JIA并MAS患儿资料,总结其临床表现、实验室检查、治疗及预后等,并选择同期住院9例未并MAS的SO-JIA患儿作为对照。结果 SO-JIA并MAS患儿关节症状多以下肢关节为主,以膝、髋、踝关节多见,上肢关节少见;多为2个或2个以上的关节受累(7例),单关节累及较少(1例);关节以大关节受累为主,小关节受累较少(1例)。浅表淋巴结大易见,肝大较脾大多见。肺高分辨率CT显示肺间质性变6例,炎性渗出5例,胸腔积液1例。MAS发作时,ALT、LDH急剧上升,WBC、中性粒细胞、RBC、CRP、ESR、纤维蛋白原(FIB)下降明显,与发病前后比较差异有统计学意义。在疾病的极期阶段,并MAS的SO-JIA患儿较非MAS的SO-JIA患儿ALT、LDH、γ-GT、CK-MB、铁蛋白明显升高,WBC、中性粒细胞、RBC、CRP、ESR、FIB明显下降。经联合方案(激素联合环孢素及丙种球蛋白)治疗,好转7例,死亡2例。结论 MAS是SO-JIA的危重并发症,激素联合环孢素及丙种球蛋白治疗可有一定的临床缓解率,早期诊断和干预有利于控制病情、改善预后,检测一些敏感指标有利于MAS的早期发现。     

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。     

幼年特发性关节炎50例临床分析及远期随访

目的探讨幼年特发性关节炎(JIA)临床特点、治疗及预后。方法总结50例JIA患儿临床表现、实验室和影像学检查结果,并随访16例患儿治疗结果,以国际风湿病学联盟(ILAR)儿科专家组的分类标准讨论稿进行分析。结果全身型8例中1例对NSAID有效;6例两药联用有效;1例加MTX,复发。JIA多关节型6例,2例对NSAID有效;3例加MTX有效;1例加CTX,复发。少关节型1例对NSAID有效。结论JIA中以全身型多见,其次为多关节型,该病临床表现多样,需根据不同临床分型予不同治疗方案,联合用药并个体化。糖皮质激素不良反应相对大,患儿耐受较差。MTX对各型均有较好疗效,主要用于多关节型以及全身型。JIA预后较好,好转率>75%。     

X-连锁无丙种球蛋白血症3例报告并文献复习

目的分析X-连锁无丙种球蛋白血症(XLA)的临床表现、诊断和治疗特点。方法回顾性分析3例XLA患儿的临床特点、细胞免疫、体液免疫指标及治疗和预后。结果 3例XLA患儿的发病年龄自11个月至6岁,中位诊断年龄为12岁。患儿均表现为多发反复细菌感染;关节炎症累及膝、踝、肘和髋等大关节。实验室检查提示血清免疫球蛋白水平及循环B细胞明显降低。3例患儿均发现存在BTK基因突变,分别为外显子3的移码突变及无义突变,外显子10的移码突变,以及外显子18的错义突变。确诊为XLA后予静脉滴注丙种球蛋白(IVIG)替代治疗;合并关节炎加用非甾体类抗炎药物(NSAIDs),酌情加用小剂量激素,病情得到明显改善。结论 XLA临床表现具有较大的变异性,反复不同部位的细菌感染,扁桃体、淋巴结发育不良及血清免疫球蛋白水平低下是早期诊断XLA的重要环节;XLA合并关节炎使用IVIG和NSAIDs联合治疗,谨慎使用激素或免疫抑制剂。     

幼年类风湿性关节炎90例

目的 分析幼年类风湿性关节炎( JRA)的临床特点和诊治措施,为JRA诊治提供参考意见.方法 收集本院2002年12月-1210年11月90例JRA患儿的临床资料,对临床表现、辅助检查、治疗方案、随访情况等进行回顾性分析.结果 1.JRA患儿90例中,男65例,女25例;年龄9个月～14岁,其中≤3岁13例,＞3～7岁23例,＞7～14岁54例.2.全身型JRA 59例(65.6％), 多关节型JRA 17例(18.9％),少关节型JRA 14例(15.5％);3种类型病例≤3岁分别为10例、2例、1例,＞3～7岁分别为18例、3例、1例,＞7～14岁分别为31例、12例、11例,不同年龄组与JRA各类型发病情况的差异无统计学意义(Pa＞0.05).3.JRA临床症状较复杂,以发热、关节肿痛、淋巴结大、皮疹为主要临床表现,全身型JRA 6例伴浆膜腔积液,4例伴肺部浸润;未发现虹膜睫状体炎.4.类风湿因子(RF)、抗核抗体(ANA)及HLA-B27阳性率分别为7.8％、8.9％和24.4％;血常规、ESR、CRP、免疫球蛋白、T淋巴细胞亚群等检查异常.5.单用非甾体类抗炎药(NSAIDs)治疗18例,72例予NSAIDs+甲氨蝶呤(MTX)或柳氮磺胺吡啶(SASP),体温控制不佳加用糖皮质激素,87例症状减轻.6.共随访26例患儿,随访5个月～6a.规则治疗组6例,无症状及关节畸形;不规则治疗20例(17例自行停药,3例不规则治疗),15例无症状,3例仍有反复发热,2例遗留关节畸形.结论 1.JRA是儿童时期较常见的与自身免疫功能紊乱密切相关的慢性关节炎,在婴幼儿、学龄前期、学龄期均可见到全身型、多关节型、少关节型.2.JRA诊断主要依靠临床特征,强调排除其他系统疾病.辅助检查用于除外其他疾病及了解疾病活动性,但无确诊意义.3.JRA应长期规范联合治疗,不规范治疗可能影响预后.     

幼年附着点炎症相关性关节炎的临床特征和治疗对策

目的交流临床诊断和治疗幼年附着点炎症相关性关节炎(ERA)的经验。方法按是否有中等．大量关节积液将患儿分成两组，根据临床表现、实验室及影像学资料总结其临床特点，并且分别用CTX MTX LEF SASP和MTX LEF SASP治疗，分别于治疗后2周、4周、8周、12周、6个月、12个月评估临床症状及ESR、CRP的变化，记录各时段患儿主观和客观的体重、血常规、肝功能、肾功能以评估药物的不良反应。结果男性有非对称性下肢外周关节炎(82．7％)、肌腱附着点炎(72．4％)、一、二级亲属有脊柱关节病史(34．5％)、骶髂关节炎(20．7％)、HLA—B27阳性(100％)是ERA的临床特征；其中有中等～大量关节积液者起病急、来势猛，临床病情评分显著大于隐匿起病者，用CTX MTX LEF SASP治疗8周后病情开始改善，12周后显著改善，改善的程度与用MTX LEF SASP治疗的非中等一大量关节积液患儿组相似，而不良反应均轻，对药物耐受性好，两组差异无显著性。结论认识ERA的临床特点，早期、积极联合使用细胞毒性免疫抑制药物治疗可获得良好的临床疗效。     

风湿病及结缔组织病

04442 0　小儿风湿病诊治进展 /王宏伟∥中国实用儿科杂志 . 2 0 0 4,19( 5) . 2 68～ 2 690 4442 1　自身抗体在风湿性疾病中的意义 /滕　庆∥中华儿科杂志 . 2 0 0 4,42 ( 4 ) . 3 15～ 3 170 4442 2　幼年类风湿性关节炎临床特征分析及治疗效果比较 /唐雪梅…∥儿科药学杂志 . 2 0 0 4,10 ( 3 ) . 2 8～2 9 ,3 4111例JRA中 ,全身型占 55% ,多关节型及小关节型占 45% ;实验室检查缺乏特异性 ;治疗上加用激素治疗者较单纯NSAIDs治疗者临床症状缓解快 ,NSAIDs 激素 MTX与NSAIDs 激素 白勺总苷治疗者比较 ,后者副作用小。表 1参 …     

儿童瑞特综合征25例

目的探讨儿童瑞特综合征（Reiter’s syndrome，RS）的发病诱因、临床特点、诊断及治疗。方法回顾性分析25例RS患儿的临床资料。治疗以非类固醇性抗炎药（NSAIDs）、柳氮磺胺吡啶、MTX及激素等为主要药物。结果25例中男21例，女4例；年龄1岁6个月～16岁。平均病程10．8周、44％的患儿发病时出现不同程度肠道症状。本组100％出现关节症状。眼炎以结膜多见（16／25）。尿道炎较为轻微，未见心脏、肾脏、肝脏等损害。本组78．2％表达HLA—B27（另2例未测）。关节症状经人院治疗15例1周内缓解，9例1个月缓解，1例未缓解。结论RS属于反应性关节炎的一种特殊类型，NSAIDs可使症状明显改善，对于慢性RS患儿，应从开始就用缓解病情抗风湿药物（DMARD）s）。     

Methotrexat in der Behandlung der juvenilen idiopathischen Arthritis

Low-dose methotrexate (MTX) is an established disease-modifying antirheumatic drug (DMARD) for the treatment of both juvenile idiopathic arthritis (JIA) and rheumatoid arthritis of adults (RA). However, in common clinical praxis, indication and application of MTX-treatment as well as monitoring of the respective side effects are differently managed. At the end of the eighties MTX was prescribed only to children, who were refractory to traditional slow-acting agents. In different clinical studies it has been found that MTX is a very safe and effective drug for treatment of JIA and that it is well tolerated in children. The common dosages of MTX in pediatric rheumatology ranges from 5–15 once a week, max. 250 mg/m²/week (0,2–0,8 mg/kg/week). The dosages given to children can be much higher than this given to RA patients (7.5–15 mg/week). This difference in dosage may be due to a different pharmakokinetic in children. Frequent side effects of MTX-treatment in children are gastrointestinal symptoms, like nausea and vomiting besides elevated liver enzymes and stomatitis. Thus, the weekly low dose MTX presents compared to other diseases-modifying antirheumatic drugs a beneficial efficiency-toxicity ratio in the treatment of juvenile idiopathic arthritis.     

Evidenzbasierter Einsatz von Methotrexat bei Kindern mit rheumatischen Erkrankungen

Juvenile idiopathic arthritis (JIA) is the most common diagnosis in children and adolescents with rheumatic disorders. JIA is successfully treated with nonsteroidal anti-inflammatory drugs (NSAR) and physiotherapy. Still, in a significant number of cases the disease is resistant to this therapy and treatment with "second line" disease-modifying antirheumatic drugs (DMARDs) is required. Methotrexate (MTX) is frequently referred to as a "first-choice second-line agent" for the treatment of JIA. However, in Germany MTX is not licensed for use in pediatric rheumatic diseases. To increase drug safety, the Working Group for Children and Adolescents with Rheumatic Diseases in Germany (AGKJR) and the Working Group on Pediatric Rheumatology in Austria have initiated the formulation of evidence-based recommendations. Based on consensus expert meetings, a MEDLINE search, standard textbooks and review articles, data from the central registry of the German Research Center for Rheumatic Diseases, experience with MTX in adults with rheumatoid arthritis (RA), and recommendations of the German Society of Rheumatology (DGRh), evidence and recommendations are graded and recommendations for the use of MTX in children and adolescents with rheumatic disease are presented.     

Moderne Behandlung der juvenilen idiopathischen Arthritis

Innovative developments in the pharmacotherapy of juvenile idiopathic arthritis (JIA) not only allowed the avoidance of long-term damage and disability but also the rapid induction of remission with shortening of the period of active disease. In this article the treatment options are discussed on the basis of clinical trials and long-term documentation of the treatment experience. While in oligoarticular JIA (oligo-JIA) intra-articular corticosteroids and non-steroidal anti-inflammatory drugs (NSAID) are initially used, patients with rheumatoid factor positive and negative polyarthritis, extended oligoarthritis (extended oligoarticular juvenile idiopathic arthritis, eoJIA) and polyarticular psoriatic arthritis (PsA) receive early treatment with disease-modifying drugs. Unless contraindicated, methotrexate (MTX) is the drug of first choice, while sulfasalazine has proven to be effective in enthesitis-related arthritis (ERA). Failure to achieve the therapeutic goal after 3–6 months prompts an escalation of pharmaceutical treatment including the use of biologics. For children above 2 years of age, the tumor necrosis factor (TNF) inhibitors etanercept and adalimumab, and the interleukin 6 (IL-6) receptor antibody tocilizumab are available. In cases of inadequate response, a change in therapy is also contemplated in short 3–6 month intervals with the aim of remission induction. In the treatment of systemic juvenile idiopathic arthritis (Still’s disease, sJIA) a distinction is made between a more systemic and articular disease patterns. While the latter is treated in the same way as polyarthritis, systemically active courses in patients with limited response to NSAIDs and corticosteroids receive an early cytokine blockade with tocilizumab or the IL-1 inhibitors anakinra or canakinumab. The use of anakinra for steroid-free first-line treatment of systemic JIA is a promising, pathophysiologically targeted therapy approach but currently still to be classified as experimental.     

Use of methotrexate in juvenile idiopathic arthritis

Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in 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。     

Use of methotrexate in juvenile idiopathic arthritis.

Methotrexate (MTX) has transformed the outlook for children with juvenile idiopathic arthritis (JIA). Most of the evidence from uncontrolled clinical trials suggests that MTX is an effective agent for treating active JIA. Data from controlled clinical trials suggests that MTX has statistically significant effects on patient centred disability measures in JIA patients with active arthritis. Although we would like a much larger study directed evidence base for our use of the drug, the studies that have been done are sound and have been followed by a change in clinical expectations and advice that speak of qualitative evidence from clinical practice, confirming the scientifically acquired data. Randomised controlled multicentre trials using sufficient numbers of patients, including functional assessment and quality of life measures, are needed to confirm the long term efficacy and safety of MTX in JIA.     

Random pharmacokinetic profiles of EC-MPS in children with autoimmune disease

Background

No consensus evidence-based guidelines for the routine laboratory monitoring of children with JIA receiving non-steroidal anti-inflammatory drugs (NSAIDs) exist. The purpose of this study is to evaluate the clinical utility of routine laboratory monitoring of hemoglobin, transaminases, blood urea nitrogen, serum creatinine, and urinalysis in patients with juvenile idiopathic arthritis (JIA) receiving NSAIDs.

Methods

The medical records of 91 children with JIA followed between 1996 and 2006 were retrospectively reviewed for laboratory results and clinically significant adverse effects attributed to NSAID use. Laboratory abnormalities were documented, with potential adverse clinical sequelae, including if NSAID use was discontinued.

Results

Abnormal laboratory results were recorded for 24 of 91 patients. Nearly all abnormalities were mild and not associated with adverse clinical sequelae. All patients but one continued to receive NSAID therapy after the abnormality was detected.

Conclusions

Although detection of abnormal laboratory values occurred while on NSAIDs, these abnormalities did not correlate with adverse clinical signs and symptoms. The routine monitoring of laboratory tests in asymptomatic children treated with NSAIDs is of questionable utility.     

Kindliches Rheuma – juvenile idiopathische Arthritis

Childhood arthralgia accounts for the most frequent symptoms leading to pediatric referral. The diagnosis of juvenile idiopathic arthritis (JIA) following a thorough assessment and differential diagnosis by the pediatrician is made in 1 among 1,000 children. On assessing the diagnosis, the specific definition of the JIA subtype (juvenile polyarthritis, oligoarthritis, systemic onset arthritis, psoriatic arthritis, and enthesitis-related arthritis) is essential to decide on the adequate therapeutic approach. DMARD treatment for JIA in recent years has been revolutionized by the introduction of biological for children with 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.