Serum lubricin levels in patients with juvenile idiopathic arthritis

ObjectivesLubricin, encoded by the proteoglycan 4 (PRG4) gene, is mainly responsible for lubricating joints. However, there is expanding evidence on its involvement in inflammatory pathways. Juvenile idiopathic arthritis (JIA) is a heterogeneous group of chronic arthritides with an unknown origin in children aged below 16 years. It is characterized by chronic joint inflammation, including synovial inflammation, and may result in cartilage destruction. We aimed to determine whether serum lubricin levels are affected in JIA patients.Material and methodsThis cross-sectional study included children diagnosed with JIA and 28 healthy controls. The patients were divided into two groups according to the presence of remission at the time of study. Lubricin protein analysis was performed by the enzyme-linked immunosorbent assay method. Serum samples were obtained at the study enrollment, and lubricin levels were measured once, and compared between JIA patients and healthy controls, and between JIA patients with active disease and remission.ResultsThe study included 52 JIA patients (28 female, 24 male) and 28 healthy controls (18 female, 10 males). The mean age at study enrollment was 11.66 ±4.41 years and 12.72 ±4.52 years in the JIA patient and control groups, respectively. Although median serum lubricin level did not differ between JIA patients (median: 0.66 ng/μl, range: 0.02–3.85 ng/μl) and healthy controls (median: 0.52 ng/μl, range: 0.06–3.84 ng/μl), it was statistically significantly higher in patients with active disease (median: 1.58 ng/μ, range: 0.08–3.85 ng/μl) than both patients in remission (median: 0.57 ng/μl, range: 0.02–3.57 ng/μl) and healthy controls. A low degree positive correlation was also found between serum lubricin levels and erythroid sedimentation rate of the JIA patients (r = 0.383 and p = 0.011).ConclusionsThis is the first study investigating serum lubricin levels in JIA patients, and we found elevated serum lubricin levels in JIA patients with active disease. Further studies are needed to clarify our results.     

Prevalence of HLA-B27 antigen in patients with juvenile idiopathic arthritis

Introduction

Human leukocyte antigen B27 (HLA-B27) is considered as a risk factor for development of juvenile idiopathic arthritis (JIA).The aim of this study was to analyse the prevalence of HLA-B27 antigen in JIA categories and its influence on disease onset and response to conventional therapy.

Material and methods

The retrospective analysis included 461 unselected children with JIA hospitalized in a single reference rheumatology centre between July 2007 and June 2012. The diagnosis was based on criteria by the International League of Association for Rheumatology. HLA-B27 was determined in 387 of all patients (84%) by hybridization of the amplified, labelled product to immobilize it on the microarray probe.

Results

HLA-B27 antigen was found in 104 of 383 affected children (27.2%), 48 of 206 girls (23.3%), and 56 of 177 boys (31.6%) – most frequently in patients with enthesitis-related arthritis (71%), psoriatic arthritis (50%) and unclassified cases (86.7%). The age of JIA onset was slightly (by 1 year) but significantly different in patients with and without HLA-B27 antigen [11 (8.5–14) vs. 10 (5–13.5) years.; p < 0.001].The use of disease-modifying antirheumatic drugs (DMARDs) and corticosteroids was more frequently clinically ineffective in HLA-B27 positive than negative patients (23.1% vs. 15.2%; p = 0.09). Patients with polyarthritis, systemic, and psoriatic arthritis more frequently received biological therapy. HLA-B27 positive patients with enthesitis-related arthritis received biological therapy more frequently than HLA-B27 negative ones (20.4% vs. 0, respectively; p = 0.09).

Conclusions

HLA-B27 antigen is a strong risk factor for the development of enthesitis-related arthritis, and to a lesser extent for psoriatic arthritis and extended course of oligoarthritis. The presence of this antigen does not affect the disease onset but seems to predict resistance to therapy with disease-modifying drugs and corticosteroids.     

Shoulder arthroplasty for patients with juvenile idiopathic arthritis

Between 1986 and 1997, 13 shoulders in adult patients who had severe polyarticular juvenile idiopathic arthritis were treated with primary arthroplasty. Eleven shoulders were evaluated retrospectively by an independent observer with a mean follow-up of 9 years. Patient evaluation included pain Visual Analogue Scale, range of motion, Disabilities of the Arm, Shoulder and Hand score, and Short-Form 36. Patients' pain decreased significantly after surgery (mean 6.7). Forward elevation improved on average by 41.1 degrees and external rotation by 39.1 degrees , without evidence of shoulder instability. Final Short-Form 36 scores and Disabilities of the Arm, Shoulder and Hand results (mean, 44.7) were poor, but all patients rated themselves satisfied with the procedure. Shoulder arthroplasty provided pain relief for end-stage shoulder involvement in adult juvenile idiopathic arthritis. Improvement in external rotation in this severely affected group appears to have a beneficial effect on functional outcome.     

COVID-19 in patients with juvenile idiopathic arthritis: frequency and severity

The aim of the study was to determine the frequency and the course of COVID-19 infection in children with juvenile idiopathic arthritis (JIA). The study involved 51 patients with JIA aged 2 to 18 years. Evidence of COVID-19 was found in 10 (19.6%) patients with JIA. COVID-19 infection occurred more often in patients with systemic arthritis (OR = 6.1667, 95% CI: 1.2053–31.5511, p = 0.0289). The course of COVID-19 infection in patients with JIA was generally similar to the course in the pediatric population, despite immunosuppressive therapy. In 3 out of 10 patients the infection caused an exacerbation of JIA, which required therapy escalation.     

Predictors of juvenile idiopathic arthritis course

Introduction

Juvenile idiopathic arthritis (JIA) is a heterogeneous group of inflammatory diseases of joints in children with various and often unfavourable prognosis. It is possible to improve the outcome of the disease in patients with JIA by a correct therapeutic choice made at disease onset – one that enables fast achievement of an inactive disease state and remission.The aim of the investigation was to develop a model/application for automatic calculation of risk of treatment-refractory JIA taking into account the combined action of clinical and cytokine factors.

Material and methods

Disease subtype was determined in 105 patients with JIA, as well as the number of poor prognostic factors and disease activity level. Blood serum cytokine levels (IL-1β, IL-4, IL-6, IL-8, IL-10, IL-17, TNF-α, IFN-γ) and their soluble receptors and agonists – interleukin 1 receptor agonist (IL-1Ra), soluble interleukin 2 receptor (sCD25), interleukin 6 (sIL6R), soluble tumour necrosis factor receptor 1 (sTNFR1) – were determined in these patients using immunoenzymatic laboratory methods.

Results

The following prognostic factors were taken into account in the study: JIA subtype, disease activity, presence of clinically unfavourable factors and cytokine characteristics. We determined that systemic subtype of JIA, moderate and high disease activity, presence of factors of poor disease course and sCD25 and IL-6 levels are statistically significant factors of treatment-refractory disease course. A Microsoft Excel application was developed for automatic calculation of risk of treatment-refractory JIA in a specific patient based on 10 factors.

Conclusions

Use of an application for automatic calculation of risk of treatment-refractory JIA enables prediction of JIA disease course in patients at disease onset and personalization of the treatment protocol.     

The use of radiosynovectomy in patients with juvenile idiopathic arthritis. Assessment of treatment efficacy and safety

ObjectivesThe aim of the study was to evaluate the usefulness of knee joint radiosynovectomy (RS) in patients suffering from juvenile idiopathic arthritis (JIA).Material and methodsOne hundred RS procedures performed in 58 patients with JIA in average age 10.4 years were evaluated.ResultsAfter 6 weeks, a decrease in the number of cases with joint pain from 90.3% to 29%, with joint oedema from 100% to 74.5%, with joint exudate from 100% to 60.6%, with gait disorders from 19.4% to 3.2%, with joint mobility disorders from 51.1% to 26.6% in the RS cases was observed. A reduction of the score in the Colorado scale from 10.9 to 4.66, in the pain visual analogue scale (VAS) from 50 to 10, in the illness VAS assessed by the patient/parent from 69.9 to 32.4, in the illness VAS assessed by the physician from 68.8 to 36.9 was observed. Six months after the RS procedure, a reduction in the number of cases with joint pain from 89.5% prior to the procedure to 29.5%, with oedema from 100% to 58.3%, with exudate from 100% to 46.9%, with gait disorders from 20% to 2.1%, with joint motility disorders from 51.1% to 26.1% was achieved. The score in the Colorado scale was reduced from 10.9 to 4.04, in the pain VAS from 40 to 0, in the illness VAS assessed by the patient/parent from 69.7 to 27.9, in the illness VAS assessed by the physician from 68.8 to 32.4. In ultrasound examinations, the greatest improvement compared to the initial condition was recorded in the 6^th month after the RS. Radiosynovectomy was positively evaluated by parents and patients in 34 anonymous surveys. Early and late observations (average 1473 days) did not show lesions at the isotope injection site, and no neoplastic lesions were observed.ConclusionsRadiosynovectomy is a valuable therapeutic option for local treatment in patients with JIA.     

人工膝关节表面置换治疗类风湿性关节炎屈膝畸形

目的探讨人工全膝关节置换术治疗类风湿性关节炎屈膝畸形的临床效果。方法选用后稳定型钴合金假体对21例类风湿性关节炎屈膝畸形患者进行人工全膝关节置换术,其中单侧膝关节置换5例,双侧膝关节置换16例,手术前后对患者的膝关节功能进行评分。结果随访1~5年,术前患者平均膝评分(22.84±6.06)分,平均功能评分(12.86±16.09)分;术后2周平均膝评分(70.16±2.71)分,平均功能评分(58.10±8.14)分;术后12周随访平均膝评分(86.14±3.12)分,平均功能评分(89.05±3.01)分。结论人工全膝关节置换术能显著纠正类风湿性关节炎屈膝畸形患者膝关节的畸形,解除疼痛,改善患膝的功能。     

类风湿性关节炎全膝关节置换的临床疗效观察

目的:观察人工全膝关节置换术治疗严重类风湿性关节炎的临床疗效.方法:我院2003年3月-2005年12月对11例严重类风湿关节炎患者行人工全膝关节置换术,平均随访13个月,按美国特种外科医院膝关节评分系统(HSS)进行疗效评定.结果:所有患者没有一例行翻修手术,X线显示所有置换假体植入位置满意,没有假体松动、移位现象出现.11个膝关节的术后情况比较满意.HSS评分平均指数从术前的41分提高到术后的90分.结论:人工全膝关节置换术可作为类风湿性关节炎患者保存关节功能,改善疼痛的一种有价值的治疗方法.     

Retrospective analyzes of adverse events during biologic agents in children with juvenile idiopathic arthritis from a single center in Turkey

ObjectivesJuvenile idiopathic arthritis is the most common rheumatic disease in childhood. Biologic agents have changed the course of juvenile idiopathic arthritis. However, there are concerns regarding the occurrence of serious adverse events in patients receiving biologic agents. The aim of this study was to evaluate adverse events in children with juvenile idiopathic arthritis receiving biologic agents.Material and methodsThis retrospective study includes juvenile idiopathic arthritis patients receiving biologic agents. Demographic features and adverse events during biologic agents were collected from medical files. Adverse events that either resulted in death, were life-threatening, required inpatient hospitalization, or resulted in persistent or significant disability/incapacity were considered as serious adverse events.ResultsIn total, 162 juvenile idiopathic arthritis patients (55.6% female) receiving biologic agents were enrolled: 101 (62.3%) patients treated with etanercept, 27 (16.7) with tocilizumab, 14 (8.6%) with adalimumab, 15 (9.2%) with anti-interleukin 1 agents (13 canakinumab, 2 anakinra), and 5 (3.1%) with infliximab. 75.9% of the patients received concomitantly disease-modifying anti-rheumatic drugs, and 20.4% received disease-modifying anti-rheumatic drugs plus corticosteroid. The mean age at initiation of the biologic agent was 10.5 ±4.3 years. The mean age at the study enrolment was 12.1 ±4.5 years. The mean follow-up duration was 19.7 ±2.1 months. The most frequent adverse event was upper respiratory tract infections (54.3%) followed by urinary tract infections (21%). Anaphylaxis occurred in 3 patients (1.9%): 2 with tocilizumab and one with infliximab. Macrophage activation syndrome occurred in 1 patient (0.6%) receiving tocilizumab. Lung tuberculosis developed in 2 patients (1.2%) receiving canakinumab. The frequency of serious adverse events in total was 6.7%.ConclusionsWhile the most frequent adverse events during biologic agents was upper respiratory tract infections, the frequency of serious adverse events was 6.7%; therefore, juvenile idiopathic arthritis patients receiving biologic agents should be carefully evaluated for these adverse events in clinical practice.     

ANCA-associated glomerulonephritis in systemic-onset juvenile idiopathic arthritis

Systemic-onset juvenile idiopathic arthritis is an inflammatory disease of unknown cause and is not commonly associated with kidney involvement. We describe 3 patients with systemic-onset juvenile idiopathic arthritis with high disease activity who developed antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis 1-6 years after the onset of systemic-onset juvenile idiopathic arthritis. Renal and systemic-onset juvenile idiopathic arthritis remission occurred in one patient under anti-interleukin 1 (anti-IL-1) treatment associated with immunosuppressive drugs. The other 2 patients developed end-stage renal disease, and one of those patients died. This report suggests that the diagnosis of ANCA-associated glomerulonephritis must be considered in patients with systemic-onset juvenile idiopathic arthritis with persistently active systemic disease who present with proteinuria. Furthermore, use of an anti-IL-1 agent might be an effective therapeutic option.     

Outcome of primary total hip arthroplasty in Charnley Class C patients with juvenile idiopathic arthritis: a case series

The outcome and complications of 37 primary total hip arthroplasties by one surgeon in 24 patients with Charnley Class C juvenile idiopathic arthritis with up to 19.6 years follow-up are reported. Twenty-six femoral components were cementless; all acetabular components were cementless with screws. Age at operation averaged 22.6 years. Two patients (3 hips) have died. Twelve hips in 9 patients have failed. Six cementless acetabular components with conventional polyethylene were revised because of osteolysis after 5.5 to 14.5 years. All 3 cementless C2 femoral stems with minimal porous coating failed. One of eight cemented AML Bantam stems loosened at 3.5 years; 2 of 23 cementless AML Bantam stems loosened at 9.5 and 19.6 years. Pain relief and functional improvement are dramatic after total hip arthroplasty in juvenile idiopathic arthritis; however, the long-term outcome is guarded.     

幼年特发性关节炎患儿身体活动的最佳证据总结

目的 总结幼年特发性关节炎患儿身体活动的相关证据,为临床医护人员提供参考。方法 提出临床问题,根据6S证据金字塔模型对幼年特发性关节炎患儿身体活动的相关证据进行检索,对纳入的文献进行质量评价,对证据进行提取及汇总。结果 共纳入13篇文献,其中包括1篇最佳实践报道、3篇指南以及9篇系统评价。汇总形成16条幼年特发性关节炎患儿身体活动的最佳证据,包括幼年特发性关节炎患儿物理治疗、身体活动时机、活动时间、活动强度、活动方式与类型、活动频率、活动场所、活动注意事项、依从性管理9个方面。结论 汇总的幼年特发性关节炎患儿身体活动最佳证据,可为医护人员指导患儿进行适量的身体活动提供循证依据。     

幼年特发性关节炎患儿报告结局评估工具研究进展

对国内外开发的幼年特发性关节炎患儿报告结局评估工具进行综述,概述幼年特发性关节炎患儿普适性和特异性自我报告结局评估工具的主要内容、应用范围、应用效果及局限性,旨在为我国幼年特发性关节炎患儿报告结局评估工具开发、患儿症状管理、精准评估以及临床决策的实施提供参考。     

幼年类风湿关节炎患儿感染水痘的临床特征

目的探讨幼年类风湿性关节炎（JIA）合并水痘患儿的临床特征。 方法收集2015年1月1日～2019年12月31日于首都医科大学附属北京地坛医院就诊的9例JIA合并水痘感染患儿（JIA合并水痘组）及同期年龄性别相匹配的18例单纯水痘患儿（单纯水痘组）的发热时间、高热比例、皮肤结痂时间、皮肤感染、血常规和肝功能及心肌酶等临床资料进行回顾性病例对照研究。 结果JIA合并水痘组患儿9例,平均年龄（6.1 ± 4.0）岁,其中男4例,女5例;有水痘接触史者8例,高热者8例,体温最高41 ℃。单纯水痘组患儿均有明确水痘接触史,高热者4例,体温最高39 ℃。JIA合并水痘组患儿发热持续时间[（7.89 ± 2.15）d）]长于单纯水痘组[（4.50 ± 1.20）d）],差异有统计学意义（t = 2.89、P < 0.001）。JIA合并水痘组患儿有典型丘疹、疱疹、痂疹,皮疹全身密集分布,疱疹数量均超过50个,以颜面最多,枕部、前胸、后背及臀部明显,大部分斑丘疹融合成片,部分融合皮疹饱满透明,黏膜可见疱疹及糜烂。而单纯水痘组患儿较少见融合性疱疹、皮肤糜烂及感染。JIA合并水痘组患儿皮疹结痂时间[（10.78 ± 2.17）d]长于普通水痘组[（5.72 ± 1.02）d],差异有统计学意义（t =-4.24、P < 0.001）。JIA合并水痘组患儿发生蜂窝织炎者4例（44.44%）,血常规粒细胞缺乏者3例（33.33%）,血小板减少者2例（22.22%）,肝功能异常者4例（44.44%）及心肌酶异常者4例（44.44%）。普通水痘组患儿中发生皮肤感染1例（5.56%）,粒细胞缺乏者1例（5.56%）及心肌酶异常者1例（5.56%）,无血小板减少和肝功能异常者。JIA合并水痘组患儿中重症出血性水痘1例。JIA合并水痘组患儿以阿昔洛韦抗病毒、大剂量静脉用人血丙种球蛋白持续治疗3～5 d,水痘皮疹给予重组人干扰素α-1b凝胶搽豆治疗,治疗期间原发病JIA未出现活动表现。普通水痘患儿给予重组人干扰素α-1b凝胶搽豆治疗。 结论幼年JIA合并水痘患儿多出现高热,且热程较长,皮肤黏膜损伤较重,出现大疱、融合甚至蜂窝织炎。治疗以阿昔洛韦抗病毒、大剂量静脉给予人血丙种球蛋白,期间未见原发病复发。     

膝关节清理术后注射透明质酸钠治疗膝骨关节炎

目的观察膝关节清理术后注射透明质酸钠治疗膝骨关节炎的疗效。方法在膝关节清理术后每月向关节腔内注射２４９ｍｍｏｌ·Ｌ－１（１％）透明质酸钠２５ｍｌ治疗膝骨关节炎３０例（３３膝）。结果术后平均随访２年,优２３膝、良７膝、差３膝。结论膝关节清理术后注射透明质酸钠治疗膝骨关节炎,可取得满意效果。     

幼年特发性关节炎累及踝部MRI表现

目的 观察幼年特发性关节炎(JIA)累及踝部的MRI表现。方法 回顾性观察42例JIA患儿受累踝部的MRI资料,分析其特点。结果 JIA共累及46侧踝部,38例单侧、4例双侧踝部受累。①37侧(37/46,80.43%)踝见关节滑膜炎,累及28个(28/43,65.12%)胫距关节、10个(10/43,23.26%)距舟关节及5个(5/43,11.63%)跟距关节;②26侧(26/46,56.52%)踝见关节积液,多累及胫距关节(19/26,73.08%),且均合并同部位滑膜炎;③26侧踝可见腱鞘炎(26/46,56.52%),多累及内踝组(15/30,50.00%)或外踝组(11/30,36.67%),前踝组较少见(4/30,13.33%),其中22侧(22/26,84.62%)合并滑膜炎、4侧(4/26,15.38%)表现为单一腱鞘炎;④15侧(15/46,32.61%)踝见骨髓水肿样改变,均累及多骨骼,常见受累部位依次为距骨(13/31,41.94%)、跟骨(10/31,32.26%)、胫骨远端(8/31,25.81%);其中11侧(11/15,73.33%)病变位于关节面下,以宽基底与关节面相连,且均合并邻近关节滑膜炎,4侧(4/15,26.67%)未伴发滑膜炎或腱鞘炎病变均远离关节面;⑤13侧踝见软组织肿胀(13/46,28.26%),分别见于内踝(7/13,53.85%)、外踝(5/13,38.46%)或内外踝同时受累(3/13,23.08%)。结论 JIA累及踝部的最主要MRI表现包括滑膜炎、关节积液和腱鞘炎,且腱鞘炎可作为唯一受累征象出现;骨髓水肿样改变常见,需与生理性表现相鉴别。     

Steroid-responsive nephrotic syndrome in a child with juvenile idiopathic arthritis

Renal disease is rare in children with juvenile idiopathic arthritis, although a number of associated nephropathies have been described, including mesangial glomerulonephritis. We report the presence of mesangial glomerulonephritis, revealed by a nephrotic syndrome, in a paediatric patient with juvenile idiopathic arthritis. Short-term steroid treatment induced a rapid remission of the nephrotic syndrome, but the presence of anti-nuclear antibodies, 1:320 in a homogeneous pattern, irregular deposits of C1q in a renal biopsy, and a mother with episodes of cutaneous lupus suggested an uncertain renal evolution for this infant.