Chronic Arthritis In Childhood Research Articles

Disease activity assessment for juvenile idiopathic arthritis in transitional care.

The indices to measure disease activity of chronic arthritis in adulthood and childhood are different. Therefore, assessing the status of the disease in young patients with juvenile idiopathic arthritis (JIA) can be tricky, especially when the transition to adult care is ongoing. The aim of our study was to assess the level of correlation between adult and juvenile scores in the measurement of disease activity in JIA patients during transitional care. We estimated the disease activity by using the Juvenile Arthritis Disease Activity Score 71 (JADAS71), clinical JADAS, adult Disease Activity Score (DAS28), Simplified Disease Activity Index (SDAI), and Clinical Disease Activity Index (CDAI) in JIA patients in transitional care. We enrolled patients older than 16 years at the time of the first transition visit, and disease activity was assessed at baseline and 12 months. Regression analyses were carried out to estimate the level of agreement among the different indices. We recruited 26 patients with JIA; 11 patients were polyarticular (42.3%) and 15 patients were oligoarticular (53.1%). The mean age at diagnosis was 7.7±3.9 years and the age at the first evaluation was 20.9±3.7 years. The correlation between JADAS71 and DAS28 was r2=0.69, r2=0.86 between JADAS71 and SDAI, and r2=0.81 between JADAS71 and CDAI. SDAI and JADAS71 showed the best correlation, but a few patients were not captured at the same level of disease activity. New prospective studies with a larger number of patients will be needed in this field.

Gene association analysis to determine the causal relationship between immune cells and juvenile idiopathic arthritis

BackgroundJuvenile idiopathic arthritis (JIA) is a type of chronic childhood arthritis with complex pathogenesis. Immunological studies have shown that JIA is an acquired self-inflammatory disease, involving a variety of immune cells, and it is also affected by genetic and environmental susceptibility. However, the precise causative relationship between the phenotype of immune cells and JIA remains unclear to date. The objective of our study is to approach this inquiry from a genetic perspective, employing a method of genetic association analysis to ascertain the causal relationship between immune phenotypes and the onset of JIA.MethodsIn this study, a two-sample Mendelian randomization (MR) analysis was used to select single nucleotide polymorphisms (SNPs) significantly associated with immune cells as instrumental variables to analyze the bidirectional causal relationship between 731 immune cells and JIA. There were four types of immune features (median fluorescence intensity (MFI), relative cellular (RC), absolute cellular (AC), and morphological parameters (MP)). Finally, the heterogeneity and horizontal reproducibility of the results were verified by sensitivity analysis, which ensured more robust results.ResultsWe found that CD3 on CM CD8br was causally associated with JIA at the level of 0.05 significant difference (95% CI = 0.630 ~ 0.847, P = 3.33 × 10−5, PFDR = 0.024). At the significance level of 0.20, two immunophenotypes were causally associated with JIA, namely: HLA DR on CD14+ CD16- monocyte (95% CI = 0.633 ~ 0.884, P = 6.83 × 10–4, PFDR = 0.16) and HLA DR on CD14+ monocyte (95% CI = 0.627 ~ 0.882, P = 6.9 × 10−4, PFDR = 0.16).ConclusionOur study assessed the causal effect of immune cells on JIA from a genetic perspective. These findings emphasize the complex and important role of immune cells in the pathogenesis of JIA and lay a foundation for further study of the pathogenesis of JIA.

Juvenile idiopathic arthritis presenting with rare extra-articular manifestations: A report of two cases

Abstract Juvenile idiopathic arthritis (JIA), a common rheumatic disease of childhood, is a consolidated term used for all chronic childhood arthritis affecting joints and extra-articular structures. Involvement of skin and internal organs such as liver, spleen, intestine, peritoneum, pleura, pericardium and myocardium will cause systemic symptoms leading to disability and mortality. Despite advanced treatment options, significant morbidity still occurs due to an unpredictable clinical course and risk of joint restriction. We describe polyarticular rheumatoid factor-negative JIA with extra-articular manifestations involving pericardium causing chronic constrictive pericarditis in a 16-year-old girl and her sibling a 13-year-old boy. The female patient developed cardiac cirrhosis, portal hypertension and oesophageal varices. Screening evaluation of this patient’s asymptomatic sibling with similar articular manifestations revealed the rare occurrence of chronic constrictive pericarditis without symptoms. Both were treated with pericardiectomy and were relieved of symptoms. Two patients of JIA from the same family presenting with constrictive pericarditis is a rare association.

Application of a mechatronic system with a stirsage sequence in magnetic resonance imaging using the whole-body protocol

The main purpose of this article is to review and analyze the conducted methodology obtained in the study of a group of patients with chronic rheumatic disease juvenile idiopathic arthritis in childhood using a new mechatronic system, using special coils and programs Survey body and Survey according to the whole-body magnetic resonance imaging protocol, evaluating the effectiveness in diagnosing inflammatory and structural changes of diseased joints compared with the routine method of studying this disease. In this paper, a new technique is considered, using an improved mechatronic system, magnetic resonance imaging for whole-body protocol research. The above research results are tabulated and analyzed. Analysis of the obtained clinical studies showed that using an additional mechatronic system with a STIRsag pulse sequence made it possible to fully assess the nature of the pathology of sections in the sagittal plane of all types of diseased joints. In the studies, a new type of laying was applied in patients with hands, instead of the standard one when the hands lie at the seams, laying was applied when the hands of the palm surface lie to the body. This made it possible to fully visualize the small wrist joints, which facilitates the examination of the patient.

18 Does overweight enhance foot disorders in patients with juvenile idiopathic arthritis?

BackgroundJuvenile idiopathic arthritis (JIA) is chronic inflammatory arthritis of childhood that may result in persistent and disabling foot impairments. Foot disorders are common in JIA with a prevalence estimated at over 90%. Many factors can worsen foot disorders in these patients. The influence of weight on foot disorders is poorly studied.ObjectivesTo examine associations between weight and foot disorders in patients with JIA.MethodsPatients with a diagnosis of JIA, based on the International League of Associations for Rheumatology (ILAR) criteria were included. Age, gender, weight, characteristics of the disease and of foot pain were noted. A specialized podiatric examination was performed.ResultsThirty-two patients were included. The mean age was 12.2 ± 2.9 [5–18]. Forty-three percent of the patients were boys (n = 14). The mean age of disease onset was 8.5 ± 3.9 [3–15]. Only one patient had a triggering factor (elbow fracture). The most common type of JIA was oligoarthritis in 12 cases, then enthesitis-related in 8 cases, polyarthritis without rheumatoid factor in 3 cases, polyarthritis with positive rheumatoid factor in 1 case, psoriatic arthritis in 3 cases, systemic arthritis in 1 case and undifferentiated arthritis in 4 cases.The mean weight was 43.5 kg [17–98]. Only 28% (n = 9) of the children had a normal weight for their age, 41% (n = 13) of them had a low weight for their age and 31% (n = 10) of them had overweight. Ten patients had foot pain: hindfoot pain in 5 patients, midfoot pain in 3 patients, and forefoot pain in 2 patients. Foot deformities were found in 78.1% of the patients (n = 27): flat foot in 39% (n = 13) of the patients and pes cavus in 39% (n = 13).There was no association between overweight and foot pain (p = 0,14) or hindfoot pain (p = 0.08). However, overweight was associated with a forefoot (p = 0.01) and midfoot (p = 0.01) pain. Overweight was not associated with foot deformities (p = 0.1).ConclusionThis study showed that weight is associated with foot pain especially forefoot and midfoot pain. A healthy lifestyle and a normal weight are fundamental to preventing foot pain at an early age in patients with JIA and improving their quality of life.

Biological classification of childhood arthritis: roadmap to a molecular nomenclature.

Chronic inflammatory arthritis in childhood is heterogeneous in presentation and course. Most forms exhibit clinical and genetic similarity to arthritis of adult onset, although atleast one phenotype might be restricted to children. Nevertheless, paediatric and adult rheumatologists have historically addressed disease classification separately, yielding a juvenile idiopathic arthritis (JIA) nomenclature that exhibits no terminological overlap with adult-onset arthritis. Accumulating clinical, genetic and mechanistic data reveal the critical limitations of this strategy, necessitating a new approach to defining biological categories within JIA. In this Review, we provide an overview of the current evidence for biological subgroups of arthritis in children, delineate forms that seem contiguous with adult-onset arthritis, and consider integrative genetic and bioinformatic strategies to identify discrete entities within inflammatory arthritis across all ages.

Biomarkers of Response to Biologic Therapy in Juvenile Idiopathic Arthritis.

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory arthritis of childhood, characterized by various clinical phenotypes associated with variable prognosis. Significant progress has been achieved with the use of biologic treatments, which specifically block pro-inflammatory molecules involved in the disease pathogenesis. The most commonly used biologics in JIA are monoclonal antibodies and recombinant proteins targeting interleukins 1 (IL-1) and 6 (IL-6), and tumor necrosis factor α (TNF-α). Several biomarkers have been investigated in JIA. Aims: To assess the level of evidence available regarding the role of biomarkers in JIA related to guiding clinical and therapeutic decisions, providing disease prognostic information, facilitating disease activity monitoring and assessing biologic treatment response in JIA, as well as propose new strategies for biologic therapy-related biomarker use in JIA. Methods: We searched PubMed for relevant literature using predefined key words corresponding to several categories of biomarkers to assess their role in predicting and assessing biologic treatment response and clinical remission in JIA. Results: We reviewed serological, cellular, genetic, transcriptomic and imaging biomarkers, to identify candidates that are both well-established and widely used, as well as newly investigated in JIA on biologic therapy. We evaluated their role in management of JIA as well as identified the unmet needs for new biomarker discovery and better clinical applications. Conclusion: Although there are no ideal biomarkers in JIA, we identified serological biomarkers with potential clinical utility. We propose strategies of combining biomarkers of response to biologics in JIA, as well as routine implementation of clinically acceptable imaging biomarkers for improved disease assessment performance.

Review of environmental factors and juvenile idiopathic arthritis.

Juvenile idiopathic arthritis is a common rheumatic disease that presents as chronic childhood arthritis. JIA is considered a multifactorial disease that may result from diverse genetic and environmental risk factors. A minority of the population-attributable risk of JIA is estimated to be due to familial factors. Thus, non-genetic or environmental factors likely account for a majority of the risk of developing JIA. Yet, while substantial data have linked environmental factors to the development of rheumatoid arthritis, similar evidence regarding JIA is sparse. This narrative review provides updates on recent literature about environmental factors that might influence the risk of developing JIA, including studies about potentially beneficial and harmful influences as well as factors with unclear effects.

Implementation of Shared Decision-Making in Clinical Practice in Rheumatology.

Implementation of Shared Decision-Making in Clinical Practice in Rheumatology.

Research progress on cardiovascular risk in juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic arthritis in childhood, and its pathological changes are similar to the inflammatory process of atherosclerosis.The traditional risk factors of cardiovascular disease include hypertension, dyslipidemia, obesity, diabetes, and so on.These risk factors may have a superposition effect on the inflammatory reaction of JIA and cause vascular endothelial dysfunction, but whether this will increase the risk of cardiovascular disease in JIA in the future, whether JIA is a new cardiovascular risk factor is still to to be confirmed. Key words: Juvenile idiopathic arthritis; Atherosclerosis; Cardiovascular disease; Risk factor

Temporomandibular joint disorders as the only manifestation of juvenile idiopathic arthritis: a case report

ABSTRACTJuvenile idiopathic arthritis is a term used to include all chronic childhood arthritis of unknown etiology. It is characterized by chronic inflammation persisting for at least 6 weeks, beginning before 16 years of age. The characteristics present are chronic synovitis, arthralgia, impaired joint mobility in at least one joint, and erosion with destruction of cartilage and subchondral bone, that could be associated or not with systemic involvement, according to each subtype of the disease. During the pathologic process, the temporomandibular joint can be involved by the juvenile idiopathic arthritis, resulting in severe mandibular dysfunction, with higher frequency in female patients. Initially, these lesions can show minor alterations like flattening of the condyle, erosions, and evolve to severe lesions, like destruction of the head of the condyle. We report a case of male patient who had destruction of both condyles, as a result from juvenile idiopathic arthritis. Proposed mechanisms to explain the juvenile idiopathic arthritis was reviewed. In this report the patient did not have pain or inflammatory process, and the temporomandibular diseases was the only manifestation.

Comparison of familial Mediterranean fever and juvenile idiopathic arthritis patients according to family origin.

Familial Mediterranean fever is an inherited condition that is more prevalent in some regions of Turkey. Juvenile idiopathic arthritis is the most common chronic arthritis of childhood. There is lack of studies on the frequency of mentioned conditions across different regions of Turkey. We aimed to compare the Familial Mediterranean fever and juvenile idiopathic arthritis patients according to their family origin. Patients with diagnosis of familial Mediterranean fever and juvenile idiopathic arthritis followed up at the Division of Pediatric Rheumatology were assessed. Data regarding the family origin of patients were noted from their medical records. Both groups were compared according to their origins. A total of 704 patients with Familial Mediterranean fever, 204 patients with juvenile idiopathic arthritis were enrolled. The main age of the patients at the study time was 12.3±4.4 years and 12.1±4.8 years, respectively. The frequency of familial Mediterranean fever was significantly higher in subjects with the origin of Black Sea and Central Anatolia regions (z score 2.69, and 3.69, respectively). Juvenile idiopathic arthritis was significantly more common in subjects from Marmara and Southeastern Anatolia regions (z score -4.11 and -2.54, respectively). The familial Mediterranean fever is more common among subjects from the Black Sea and Central Anatolia regions of Turkey, especially Kastamonu, Sivas and Tokat provinces. Whereas, patients with juvenile idiopathic arthritis more commonly originate from Marmara and Southeastern Anatolia regions of Turkey.

Noninflammatory disorders mimic juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common chronic childhood arthritis; unfortunately, no diagnostic tool is available. Genetic disorders with musculoskeletal involvement that mimic chronic polyarthritis should be considered in the differential diagnostics of JIA. Normal inflammatory markers and characteristic radiological features are able to distinguish these disorders from JIA. Timely diagnosis of these disorders is crucial to offer the family proper genetic counseling and avoid inappropriate therapy. This review highlights selected noninflammatory disorders that often present with articular manifestations and that are often mislabeled as JIA. The focus is on the clinical, biochemical, and imaging features of these disorders.

Are We Right to Consider Mesenchymal Stem Cells to Be a New Perspective for Patients with Juvenile Idiopathic Arthritis?

Juvenile idiopathic arthritis (JIA) is the most common cause of chronic arthritis in childhood. Up to 50% of patients are resistant to standard therapy, which includes non-steroid anti-inflammatory drugs, corticosteroids, disease-modifying anti-rheumatic drugs and biologic therapies. Intra-articular injection of mesenchymal stem cells (MSCs) is proposed as a new approach to JIA treatment. MSCs can modulate inflammation via mechanisms of both adaptive and innate immune response. They are able to inhibit T and B cell proliferation, promote regulatory T cells, suppress the maturation of dendritic cells, stimulate macrophage differentiation into M2 phenotype and reduce effectiveness of natural killer cells. They also secrete plethora of soluble factors which influence joint inflammation. Recent clinical studies reviewed in the article provide promising results which may suggest including intra-articular injection of MSCs in therapy of patients with oligoarticular JIA.

Study on clinico-serological profile of juvenile idiopathic arthritis in children

Background: Chronic arthritis, the most common chronic rheumatic disease of childhood, is one of the more frequent chronic illnesses of children. Besides clinical examination, various hematological and immunological markers are being used to diagnose Juvenile Idiopathic Arthritis (JIA). The present study was designed to be conducted in our institution to find out the clinico-serological profile of JIA. Materials and Methods: All children under 14 years, attending the OPD and suspected to be suffering from Juvenile Idiopathic Arthritis, during September 2010 to September 2012 were included in the study. They were screened for the diagnosis of JIA. All the cases were subjected to hematological, immunological, biochemical and radiological investigation. Analysis was made on clinico-serological profile of the disease. Results: Majority out of 40 cases belonged to the age group 5-10 years and males out-numbered the females. Pallor was present in 100% of SOJIA and Polyarticular RF +ve JIA, 78.5% in Polyarticular RF –ve JIA, and 60% of Enthesitis related arthritis (ERA). In systemic onset JIA, soft tissue swelling was found in 66.67% cases. No cases of psoriatic arthritis or undifferentiated types were detected. Juxta articular osteoporosis was found in 66.67% cases. Methotrexate, Oral steroid and Hydroxychloroquine were the drugs used for most of the varieties of JIA. Conclusion: Even though, different varieties of JIA were detected, no cases of psoriatic arthritis or undifferentiated types were found. Chronic arthritis in childhood shows very diverse clinical pattern. JIA is like an iceberg, the diagnosis of which is usually missed due to lack of definite diagnostic parameters and less accessibility of the common man to the health facility.

Validation of administrative case ascertainment algorithms for chronic childhood arthritis in Manitoba, Canada.

We validated case ascertainment algorithms for juvenile idiopathic arthritis (JIA) in the provincial health administrative databases of Manitoba, Canada. A population-based pediatric rheumatology clinical database from April 1st 1980 to March 31st 2012 was used to test case definitions in individuals diagnosed at ≤15years of age. The case definitions varied the number of diagnosis codes (1, 2, or 3), time frame (1, 2 or 3years), time between diagnoses (ever, >1day, or ≥8weeks), and physician specialty. Positive predictive value (PPV), sensitivity, and specificity with 95% confidence intervals (CIs) are reported. A case definition of 1 hospitalization or ≥2 diagnoses in 2years by any provider ≥8weeks apart using diagnosis codes for rheumatoid arthritis and ankylosing spondylitis produced a sensitivity of 89.2% (95% CI 86.8, 91.6), specificity of 86.3% (95% CI 83.0, 89.6), and PPV of 90.6% (95% CI 88.3, 92.9) when seronegative enthesopathy and arthropathy (SEA) was excluded as JIA; and sensitivity of 88.2% (95% CI 85.7, 90.7), specificity of 90.4% (95% CI 87.5, 93.3), and PPV of 93.9% (95% CI 92.0, 95.8) when SEA was included as JIA. This study validates case ascertainment algorithms for JIA in Canadian administrative health data using diagnosis codes for both rheumatoid arthritis (RA) and ankylosing spondylitis, to better reflect current JIA classification than codes for RA alone. Researchers will be able to use these results to define cohorts for population-based studies.

Atypical monoarthritis presentation in children with oligoarticular juvenile idiopathic arthritis: a case series

BackgroundOligoarticular juvenile idiopathic arthritis (oligoJIA), the most common chronic inflammatory arthritis of childhood, usually involves the knees and ankles. Severe oligoJIA monoarthritis presenting in a joint other than knees and ankles, is rare.FindingsWe report four children who presented with severe isolated arthritis of the hip, wrist or elbow and were diagnosed with oligoJIA. All four were girls with a median age of 11.5 years. Those with hip arthritis also met the classification criteria for juvenile-onset spondylarthopathy. Median duration of symptoms prior to diagnosis was 9.5 months. Three children had already cartilage loss or erosive disease at diagnosis.ConclusionsChildren diagnosed with oligoJIA that present with monoarthritis of the hip, wrist and elbow can have aggressive disease. Girls with positive HLA-B27 presenting with isolated hip arthritis could meet the classification criteria for both oligoJIA and juvenile-onset SpA. Early referral to specialized care may improve their diagnosis, treatment and outcome.

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications

ObjectivesJuvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of...

Management of Children with Juvenile Idiopathic Arthritis.

Juvenile idiopathic arthritis (JIA) comprises a group of heterogeneous disorders of chronic arthritis in childhood and remains the commonest pediatric rheumatic disease associated with significant long-term morbidity. Advances in understanding of the pathogenesis, better definition of disease control/remission measures, and the arrival of biological agents have improved the outcomes remarkably. Methotrexate (Mtx) remains the first-line disease modifying (DMARD) therapy for most children with JIA due to its proven efficacy and safety. Sulphosalazine (SSz) (especially for enthesitis) and leflunomide may also have a secondary role. Tumor necrosis factor inhibitors (TNF-I), alone or in combination with Mtx have shown tremendous benefit in children with polyarticular JIA, enthesitis related arthritis (ERA) and psoriatic arthritis. Tocilizumab appears very efficacious in systemic arthritis and abatacept and tocilizumab also appear to benefit polyarticular JIA; the role of rituximab remains unclear, though clearly beneficial in adult RA. TNF-I with Mtx is also effective in uveitis associated with JIA. Biologicals have demonstrated an impressive safety record in children with JIA, although close monitoring for rare but potentially dangerous adverse events, such as tuberculosis and other infections; paradoxical development of additional autoimmune diseases; and possibly an increased risk of cancers is warranted.

Enthesitis-related arthritis.

Juvenile idiopathic arthritis (JIA) is the most common chronic arthritis of childhood. Currently, it is characterized by seven categories. The enthesitis-related arthritis (ERA) category usually affects boys older than 6 years and presents with lower limb asymmetrical arthritis associated with enthesitis. Later, these children can develop inflammatory lumbosacral pain (IBP). These children are at risk of developing acute anterior uveitis. A recently devised disease activity index, Juvenile Spondyloarthropathy Disease Activity Index (JSpADA), has been validated in retrospective cohorts. The corner stone of treatment is NSAIDs, local corticosteroid injections, and exercise. Methotrexate and sulfasalazine can be used for peripheral arthritis while anti-tumor necrosis factor (TNF) agents are sometimes used to treat refractory enthesitis and sacroiliitis. Almost two third of patients with ERA have persistent disease and often have impairments in their quality of life. The presence of hip or ankle arthritis and a family history of spondyloarthropathy or polyarticular joint involvement at onset are associated with poorer prognosis.