�����ϰ����Ŀ����������ط��Թؽ�������е�����

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。     

Anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis

Objective  

Prevalence and clinical significance of anti-cyclic citrullinated peptide (CCP) antibodies in Indian patients with juvenile idiopathic arthritis (JIA).     

4种自身抗体在幼年特发性关节炎早期诊断和预后评估中的意义

目的探讨免疫学指标抗角蛋白抗体(AKA)、抗环瓜氨酸肽(抗CCP)、类风湿因子(RF)-IgG、RF-IgM与各分型幼年特发性关节炎(JIA)患儿早期诊断和预后之间的关系。方法收集76名经系统治疗并坚持随访治疗至少6个月的JIA患儿,在诊断初期进行AKA、抗CCP、RF-IgG、RF-IgM等免疫指标的检测,对不同亚型和预后作阳性检出率的比较,进行敏感性、特异性、相关危险度的统计学分析,并与49例健康儿童作对照比较。结果多关节炎型组的AKA、抗CCP、RF-IgG、RF-IgM的阳性检出率与正常对照组比较差异有统计学意义,全身型、少关节型、附着点炎相关型的自身抗体阳性检出率与正常对照组差异无统计学意义。4种抗体的检测对多关节炎型组的分型敏感性依次为AKA>抗CCP,RF-IgG>RF-IgM;特异性依次为RF-IgM>AKA>RF-IgG>抗CCP。难治型JIA患儿的AKA阳性检出率与普通JIA患儿差异有统计学意义;相对危险度OR为3.514。结论 AKA、抗CCP、RF-IgG、RF-IgM在JIA不同亚型早期诊断中的作用有区别;AKA、抗CCP抗体的检测对JIA多关节炎型有较好的敏感性和特异性;AKA抗体...     

抗环瓜氨酸肽抗体和隐匿性类风湿因子免疫球蛋白M型在诊断早期幼年类风湿关节炎中的意义

目的 检测抗环瓜氨酸肽(CCP)抗体及隐匿性类风湿因子IgM型(HRF-IgM),并探讨其在幼年类风湿关节炎(JRA)早期诊断中的临床意义。方法 用人工合成CCP链为抗原检测抗CCP抗体;对27例早期诊断的JRA做动态检测,通过阳性预测值(PPV)和阴性预测值(NPV)确定抗CCP抗体和HRF-IgM对早期诊断的JRA的特异性和敏感性。结果 抗CCP抗体和HRF-IgM总阳性率分别为58.5％、65.0％。后者敏感性要高于前者,病情越重或受累的关节越多,抗体检出率越高。对早期JRA的PPV、抗CCP抗体特异性要高于HRF-IgM。当两种实验联合应用时,对具有早期关节炎表现发展成JRA的PPV为93.7％。结论 抗CCP抗体和HRF-IgM在JRA患儿均有较高的检出率,并与疾病严重程度有关。抗CCP抗体与HRF-IgM联合应用时,可使JRA的PPV进一步提高。     

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.     

Inflammation in juvenile idiopathic arthritis

Inflammation evolved to aid in the clearance of microorganisms. In pediatric arthritides, the inflammation persists and causes damage to the joint. The contribution of the innate immune system to inflammation is significant and can be exploited therapeutically. Although cells of the adaptive immune system such as T cells and B cells participate in the disease process, many of the features of arthritis are directly attributable to inflammatory mediators. Recent advances in the understanding of these processes have led to dramatic improvements in treatment.     

Uveitis in juvenile idiopathic arthritis

OBJECTIVE: To evaluate the frequency of chronic anterior uveitis in patients with juvenile idiopathic arthritis and its association with the presence of antinuclear antibodies. PATIENTS AND METHODS: We retrospectively studied 72 patients with juvenile idiopathic arthritis. All of them were submitted to slit-lamp examination of the anterior chamber at diagnosis. Both antinuclear antibodies and rheumatoid factor were determined. Patients with positive results for antinuclear antibodies were evaluated every three months and those with negative results were assessed every six months. RESULTS: Forty patients were male (55.5%) and 36 were Caucasoid (50%). The mean age at the onset of juvenile idiopathic arthritis was 6.4 years (range = 1 to 14 years) and the mean age at the beginning of the study was 10.4 years (1 to 19 years). According to the type of disease at onset, 32 were pauciarticular (44.4%) (17 boys and 15 girls), 30 were polyarticular (41.6%) (17 boys and 13 girls) and 10 were systemic (14%) (6 boys and 4 girls). We observed chronic anterior uveitis in five patients (6.5%) (mean age = 11.4 years). Among them, four (80%) had pauciarticular juvenile idiopathic arthritis at disease onset (three girls with type I juvenile idiopathic arthritis and positive antinuclear antibodies and one boy with type I juvenile idiopathic arthritis and negative antinuclear antibodies) and one girl with polyarticular juvenile idiopathic arthritis (negative antinuclear antibodies and rheumatoid factor). In this group, the mean age at the onset of juvenile idiopathic arthritis was 5.1 years and the mean age of uveitis onset was 9 years. Antinuclear antibodies were positive in 3/5 patients (60%) with uveitis. Antinuclear antibodies were positive in 12% of the patients without uveitis (n = 67). Among the patients with uveitis, three had only one flare and the other two had four flares with cataract. The frequency of antinuclear antibodies was statistically higher in the patients with uveitis (P< 0.05). CONCLUSION: Although the incidence of uveitis in our study was lower than that reported in the literature, the frequency of uveitis was higher in females, in those with pauciarticular juvenile idiopathic arthritis and in patients with positive antinuclear antibodies.     

Diagnosing juvenile idiopathic arthritis

Inflammatory arthritis in children and young people is a disease of unknown aetiology with a significant burden in terms of morbidity and disability. Prompt diagnosis is essential as effective treatments are available. The term juvenile idiopathic arthritis (JIA) has been widely adopted to describe this heterogeneous group of diseases, following the classification system published by the International League of Associations for Rheumatology (ILAR). This is an attempt to unite the historical gap between American and European terminology in which the terms juvenile rheumatoid arthritis and juvenile chronic arthritis, respectively, were used. Standardized diagnosis has improved comparability of research and in turn has enhanced the evidence base in JIA.The diagnosis of JIA is predominantly clinical and this review outlines the clinical features of the condition together with an approach to differential diagnosis. The role of the limited laboratory testing is reviewed, along with recent advances in imaging which can aid diagnosis. Of particular importance are joint ultrasound, which has the potential to be used routinely in the consulting room as part of a clinician’s assessment, and MRI that can provide early diagnostic clues to 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.     

托珠单抗治疗全身型幼年特发性关节炎的疗效和安全性

幼年特发性关节炎(juvenile idiopathic arthritis,JIA)是最常见的儿童风湿性疾病之一.全身型幼年特发性关节炎(systemic juvenile idiopathic arthritis,SJIA)是JIA的一个亚型,其发病机制可能有别于其他亚型.已有研究表明,IL-6、IL-18、IFN-γ而非TNF-α在SJIA中起主要作用.传统的生物制剂如TNF拮抗剂对SJIA疗效有限.托珠单抗(tocilizumab,TCZ)是一种人源化抗人白细胞介素-6受体(IL-6R)的单克隆抗体,通过抑制IL-6与跨膜型IL-6R或可溶型IL-6R的结合,阻断IL-6介导的炎性反应和关节破坏.该文就TCZ治疗SJIA的疗效和安全性研究进展作一综述.     

Growth of children with juvenile idiopathic arthritis

Objective

To evaluate the growth pattern in children with juvenile idiopathic arthritis and its subtypes in comparison with age, sex and temporally matched controls.

Study design

Prospective study.

Setting

Pediatric rheumatology clinic of a tertiary care hospital in Eastern part of India.

Participants

Seventy-five children (2–12 years) diagnosed as juvenile idiopathic erthritis by International League of Associations for Rheumatology criteria and 75 age- and sexmatched controls.

Intervention

Weight, height and body mass index were recorded at six monthly interval in both groups over a period of 3 years.

Main outcome measures

weight, height and body mass index.

Results

Subtype distribution of juvenile idiopathic arthritis was: oligoarthritis (49%, n=37), rheumatoid factor negative polyarthritis (27%, n=20), rheumatoid factor positive polyarthritis (8%, n=6), systemic onset (15%, n=11) and enthesitis related arthritis (1.3%, n=1). Anthropometric parameters in children with juvenile idiopathic arthritis were not significant different from controls. Comparison between the subtypes showed significant differences in height (P=0.011), weight (P=0.005), and growth velocity (P=0.005), but not in body mass index. Systemic onset disease led to significant restriction in height (P=0.018; 95% CI 2.13–33.77) and weight (P=0.008; 95% CI 1.47–14.43) compared to controls. Growth velocity was significantly affected in rheumatoid factor positive polyarthritis (P=0.003; 95% CIO. 46–3.14).

Conclusions

Children with juvenile idiopathic arthritis do not have significantly lower values of anthropometric parameters compared to controls. Significant restriction in height and weight is seen in systemic onset disease, and growth velocity is significantly reduced in rheumatoid factor positive subjects.     

Management of juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is a relatively uncommon disorder in childhood. Expertise however should be the corner stone of care of children with JIA, as early appropriate treatment is mandatory to ensure best possible short and long-term outcome for children with JIA. Therefore comprehensive treatment centers (with multi disciplinary teams) should be based in tertiary level academic centers. This article deals with both specific and generic issues encountered in managing children with JIA.