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Whole body bone scan using technetium-99m shows increased bone uptake in the lesions of a patient with juvenile idiopathic arthritis.
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Juvenile idiopathic arthritis (JIA) is comprised of a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and has symptoms lasting at least 6 weeks. The previous classification of JIA included seven different categories, whereas its current classification was compiled by the In...
Citations
... Juvenile idiopathic arthritis (JIA) is a rheumatologic disease characterized by enduring, chronic inflammation, presenting before the age of 16, the precise cause of which remains unknown [1]. The International League of Associations for Rheumatology (ILAR) uses the term JIA as an inclusive term, instead of the previously used terms juvenile chronic arthritis and juvenile rheumatoid arthritis [2,3]. ...
... JIA necessitates vigilant monitoring in children to assess potential joint damage, extra-articular organ-system damage, and other associated medical problems [1]. Patients undergo numerous medical evaluations during and following the diagnosis of JIA [1,6]. ...
... JIA necessitates vigilant monitoring in children to assess potential joint damage, extra-articular organ-system damage, and other associated medical problems [1]. Patients undergo numerous medical evaluations during and following the diagnosis of JIA [1,6]. Apart from the direct ramifications of the disease, individuals may also experience adverse effects from treatments [7]. ...
Introduction This study aimed to assess the cultural adaptation, validity, and reliability of the Turkish version of the Juvenile Arthritis Quality of Life Questionnaire (JAQQ) in patients with juvenile idiopathic arthritis (JIA).
Methods A total of 100 JIA patients (64% female), aged 9 to 18 years, participated in the study conducted at a tertiary care university hospital. The JAQQ was culturally adapted through a rigorous translation process and administered alongside established measures, including the Childhood Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Biopsychosocial Questionnaire (JABQ), and Children's Depression Inventory (CDI). Validity and reliability were evaluated using Spearman's correlation coefficients, Cronbach's alpha, intraclass correlation coefficient (ICC), standard error of the mean (SEM), and minimal detectable change (MDC).
Results The Turkish version of JAQQ exhibited high convergent validity, correlating significantly with CHAQ, JABQ, and CDI. No floor or ceiling effects were observed in the total JAQQ score, indicating a balanced assessment. Internal consistency was excellent (Cronbach's α = 0.948), and test-retest reliability was satisfactory (ICC = 0.913). SEM and MDC 95 values were 0.357 and 0.99, respectively.
Conclusions The Turkish adaptation of JAQQ emerges as a valid and reliable instrument for comprehensively assessing the health-related quality of life in children and adolescents diagnosed with JIA. The questionnaire's robust psychometric properties, coupled with distinctive features like individualized assessment, highlight its potential as a valuable tool for both clinical assessment and scientific research in the field of pediatric rheumatology.
Key Points
• The Juvenile Arthritis Quality of Life Questionnaire (JAQQ) is an important scale that evaluates the quality of life of children with Juvenile Idiopathic Arthritis (JIA).
• JAQQ is known and used in the field of pediatric rheumatology in Turkey, but its Turkish adaptation has not been made before.
• Our study includes 100 JIA patients aged between 9 and 18 years and shows that the Turkish version of JAQQ is valid and reliable in measuring the quality of life of these children.
• This research contributes to the accurate assessment of the quality of life in Turkish children diagnosed with JIA, providing valuable insights for both clinical and scientific studies.
... Unlike RA, a single disease, JIA has been defined as an umbrella term covering 7 different types of childhood chronic arthritis. [25] The current PRINTO discussion of reclassification of JIA is ongoing, but poly and oligoarticular JIA will continue to be the most common subtypes according to available criteria. [5] In juvenile idiopathic arthritis, patients could present erosions and deforming patterns of different joints during their illness. ...
Introduction
The term “Rhupus” was employed to descriptively illustrate the overlap observed in some pediatric patients displaying features of both juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE). Although “Rhupus” is traditionally used in adults, we applied it broadly to emphasize this clinical overlap.
Methods
We sought to identify studies that registered signs, symptoms, imaging characteristics, and treatments given to patients with JIA and SLE. We searched four databases using a Boolean search string, resulting in 231 articles after duplicate removal. Title and abstract screening yielded 57 articles for full-text assessment. Full reviewed 13 extracted data regarding sex, age of onset, serologic and imaging findings, and management strategies. The NIH quality assessment tool was applied to ensure the internal validity of the articles.
Results
From the 13 articles evaluated that meet inclusion criteria, none had standardized diagnostic algorithms. The total number of patients in those articles is 26, without discussing treatment guidelines.
Discussion
Clinical presentation, diagnostic parameters, and treatment of pediatric Rhupus were synthesized in this review. Fundamental keys help distinguish the joint presentation when Juvenile Idiopathic Arthritis or Lupus is present, compared with the signs and symptoms when developing the overlapping syndrome. We highlight the importance of physicians knowing about this rare condition and call all specialists to report new cases of the disease so a consensus can be reached to establish standardized guidelines for diagnosing and treating Rhupus syndrome.
... Another very common chronic disease is juvenile idiopathic arthritis (JIA), which is a chronic condition characterized by persistent joint inflammation for at least 6 weeks, with an onset before the age of 16 years and an unknown cause [40], and it is the commonest rheumatic disease in children [41]. The symptoms of JIA can vary widely but commonly include persistent joint pain, swelling, and stiffness, which can lead to functional impairment and reduced quality of life [42]. The etiopathogenesis of JIA is complex and multifactorial, with environmental factors being considered relatively controllable determinants [43]. ...
Chronic diseases are a growing problem for global health due to the large number of people they involve, the repercussions they have on the mental and physical well-being of those affected, and the costs to society. Particularly, chronic illnesses of childhood have important psychological implications, not only for affected children but also for their parents. Among these pathologies, neurodevelopmental disorders (NDDs) and uveitis associated with juvenile idiopathic arthritis (JIA-U) may affect mental and physical health, emotions, memory, learning, and socializing. This study evaluates the psychological and behavioral/emotional impact of NDDs and JIA-U on children and parents. Specifically, 30 children with active JIA-U and 30 children with NDDs and their parents completed the Child Behavior Checklist (CBCL) and Parent Stress Index—Short Form (PSI) questionnaires. Children with NDDs have statistically significant differences in all the emotional and behavioral variables compared to JIA-U children, and parents of children with NDDs experience an increased stress load compared to parents of children with JIA-U. This study emphasizes the wide range of emotional and behavioral challenges that parents face with NDDs. This study emphasizes that parents of children with NDDs not only experience higher levels of stress compared to parents of normally developing children but also experience higher levels of stress compared to parents of children with potentially debilitating chronic diseases such as JIA-U.
... In pediatric settings, the spectrum of potential arthritis diagnoses can be relatively broad, primarily including infectious and inflammatory causes and, to a lesser extent, oncological diseases [34][35][36][37]. With respect to inflammatory causes, distinguishing between JIA and other types of arthritis can prove to be a challenge, mainly because of the lack of specific antibodies in several JIA subtypes [35,38]. ...
... In pediatric settings, the spectrum of potential arthritis diagnoses can be relatively broad, primarily including infectious and inflammatory causes and, to a lesser extent, oncological diseases [34][35][36][37]. With respect to inflammatory causes, distinguishing between JIA and other types of arthritis can prove to be a challenge, mainly because of the lack of specific antibodies in several JIA subtypes [35,38]. Over time, research has focused on investigating potential markers to assist clinicians in discriminating arthritis patients more accurately [13,39]. ...
In pediatric care, the range of potential diagnoses for arthritis can be relatively extensive, primarily involving infectious and inflammatory causes and, to a lesser extent, oncological conditions. Specifically, when addressing inflammatory causes, differentiating between Juvenile Idiopathic Arthritis (JIA) and Reactive Arthritis (ReA) can prove to be challenging during the first weeks, owing to the lack of specific antibodies in several JIA subtypes. This single-center retrospective study of 108 children with arthritis aimed to evaluate in greater detail the complete blood count (CBC) profiles of children with JIA and ReA in greater detail. The most significant differences were noted in terms of the Systemic Immune-Inflammation Index (SII), with higher values in the JIA group. Moreover, within the JIA group, SII displayed a significant positive correlation with conventional inflammatory biomarkers, specifically C-reactive protein (ρ = 0.579) and Erythrocyte Sedimentation Rate (ρ = 0.430). It was the only independent factor associated with the presence of JIA after adjusting for age (p = 0.030). Also, even with the moderate diagnostic value, the discriminating capacity of SII was superior to those of each of its component CBC parameters according to receiver operating characteristic (ROC) analysis. In summary, this study identified elevated SII values in the JIA group compared to the ReA group, indicating the potential utility of SII as an adjuvant discriminatory marker between these two arthritis forms.
... Twenty patients less than 16 years old with ILARdiagnosed JIA were enrolled in this prospective cohort research at Tanta University Hospitals' (5) , Erythrocyte sedimentation rate (ESR) (6) , C-reactive protein (CRP). (7), Serum ferritin. ...
... The JIA is known to be a heterogeneous inflammatory disease and described as "arthritis lasting for 6 weeks or longer with onset before 16 years of age with no known reason". 1 Significant degree of morbidity and disability is associated with JIA which negatively influence the quality of life and routine activities. 2 Variation is observed in terms of prevalence of JIA is different parts of the world and races. 3 The estimated incidence of JIA is calculated to be between 2 to 20/100,000 population while it is calculated to be between 7 to 400 per 100,000 in the pediatric age groups. ...
Objective: To determine the frequency of different types of juvenile idiopathic arthritis (JIA) in children presenting in a tertiary care hospital. Study Design: Cross-sectional study. Setting: Department of Pediatrics, Children Hospital Complex & Institute of Child Health Multan. Period: January 2021 to July 2021. Material & Methods: Children of both genders with diagnosis of JIA aged 6-12 years and having duration of illness more than six weeks. Age, gender and duration of JIA were noted. Stratification of confounder variables e.g., age, gender and duration of JIA was done. Post-stratification Chi-square test was applied to determine the effect of the variables on frequency of different forms of arthritis. P-value <0.05 was taken as significant. Results: In a total of 121 children, mean age was 8.84±1.83 years. 72 (59.5%) were boys. Mean duration of symptoms was 7.93±4.61 months. Oligoarticular JIA was most common type diagnosed in 40 (33.1%) children followed by polyarticular JIA in 37 (30.6%). Oligoarticular and polyarticular JIA were significantly more common in boys (67.5% and 67.6%) compared to girls (32.5% and 32.4%) respectively (p<0.001). Conclusion: Oligoarticular JIA sub-type is the most common subtype of JIA. Identification of these different subtypes is beneficial for diagnosis and long-term treatment of such children.
... Haemoleucogram can show anaemia associated with low serum iron, which is usually attributed to the chronic disease. Children with active disease can present leukocytosis and increased platelet count (Kim & Kim, 2010). ...
... Other laboratory tests include serology for the presence of anti-nuclear antibody (ANA), particularly for the patients with oligoarticular onset disease and anti-cyclic citrullinated peptide (anti-CCP) antibodies, which may indicate severe disease. Children that have symptoms of enthesitis-related arthritis should be tested for HLA-B27 (Kim & Kim, 2010). ...
... Conventional radiology and ultrasonography are often used for affected joints evaluation or for intra-articular fluid identification. Joint assessment in JIA is also done by standard magnetic resonance (MR) imaging, but unfortunately there is no imaging modality that can alone confirm the diagnosis (Kim & Kim, 2010). ...
... To estimate overall immunological status and potential disease severity, the concentrations of serum immunoglobulins (IgG, IgA, and IgM), anti-nuclear antibody (ANA), and complements (particularly C3 and C4) have also been determined. 72 Depending on the severity of the disease, JIA has been treated with intra-articular glucocorticoid (triamcinolone hexacetonide) joint injections, methotrexate, and biological agents (TNF inhibitor, IL-6 and anti-IL-1 receptor antagonists). 73,74 The current biological treatments modulate the specific mechanisms of the immune systems, such as T-and B-lymphocyte activation and/or functioning, or cytokines (TNF-α, IL-1 and IL-6) signaling depending on their sites of actions ( Figure 3). ...
For unknown reasons, the incidences of many chronic pediatric autoimmune
diseases have been increasing in the past decade, placing long-term burdens on
families and healthcare systems. To prevent the rising numbers of these diseases, it
is important to find their potential risk factors.
The general aim of this study was to search for mutual environmental factors that
might contribute to overall development of pediatric autoimmune diseases,
represented by type 1 diabetes (DM), autoimmune thyroiditis (AIT), juvenile
idiopathic arthritis (JIA), and inflammatory bowel diseases (IBD). These diseases
were chosen due to their chronic nature and similarities in their pathogenesis. The
specific aim was to estimate the likelihood of developing autoimmune diseases after
exposures to potential environmental risk factors at different stages of childhood.
This register-based longitudinal study utilized The Finnish Health in Teens (FinHIT) cohort – a nationwide prospective cohort to address health behaviors of
Finnish children and adolescents, comprising over 11,000 children (born 2000-2005)
without specific exclusion criteria. The participants were recruited through schools,
and baseline data collection was performed in 2011–2014. At school, the participants
were asked to answer a questionnaire on their dietary habits, and their height, weight,
and waist circumference were measured in a standardized way by trained
fieldworkers.
The children in the Fin-HIT cohort were followed-up for a median of 16.6 years
through national health registers. Data on autoimmune diagnoses (DM, JIA, and
IBD) from birth until the end of the follow-up were obtained from the Special
Reimbursement Register, and data on thyroxin (as an indicator for AIT diagnosis)
and outpatient antibiotic purchases were obtained from the Drug Purchase Register.
Both of these registers are maintained by the Social Insurance Institution (i.e KELA).
Maternal and perinatal data were obtained from the Medical Birth Register
maintained by the Finnish Institute for Health and Welfare.
By the end of the follow-up, 245 (2.2%) children received primary diagnosis, of
which 102 were DM, 61 were AIT, 54 were JIA, and 28 were IBD. Nine children
also had a secondary diagnosis (7 were AIT, 1 was JIA, and 1 was IBD), comprising
a total of 254 diagnoses. The incidence for each diagnosis per 100,000 children/year
were 106.1 for DM, 46.0 for AIT, 55.0 for JIA, and 23.7 for IBD.
Children who developed studied autoimmune diseases were compared with those
who did not. The analyzed risk-factor variables were: (1) maternal and perinatal
factors (including gestational age and mode of delivery); (2) frequency and types of
antibiotic exposures throughout childhood; and (3) dietary patterns (including eating
habits and the consumption frequencies of sugary products, fruit, and vegetables),
overweight (based on BMI categories), and central obesity (present when weight to
height ratio was ≥0.5) of school-aged children. The likelihood of developing an
autoimmune disease was estimated using Odds Ratio (OR).
Preterm birth (<37 weeks) was more common in children with autoimmune
diseases than in those without (8.6% vs. 5.3%, p=0.035), and was not associated with
any particular diagnosis in this study. Cesarean section was not related to the
development of autoimmune diseases, nor was number of antibiotic exposures
throughout childhood (from birth to the age of diagnosis/ corresponding age for
matched controls). Macrolides showed minor association with autoimmune diseases
(OR 1.24, 95% CI 1.01-1.51) when exposures occurred within 2 years before
diagnosis. In contrast, exposures to penicillins at any stages of childhood were not
related with the onset of any autoimmune diseases, despite of their frequent use
(approximately 40% of all antibiotics). Furthermore, eating habits or consumption
frequency of sugary products, fruit, and vegetables at baseline and before diagnosis
did not differ between cases and controls. Conversely, central obesity in school-aged
children increased the likelihood of developing autoimmune diseases later in
adolescence (OR 2.11, 95% CI 1.11-3.98).
Conclusions: Preterm birth, exposures to macrolides, and central obesity in schoolaged children were identified as shared risk factors for pediatric autoimmune diseases
in this study. The detailed mechanism on how these potential risk factors are related
to the development of autoimmune diseases warrants further studies.
... Another autoimmune disorder of positive association with IgAD is juvenile idiopathic arthritis (JIA) [5]. According to the International League of Associations for Rheumatology, JIA comprises a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and with symptoms lasting for at least 6 weeks [6,7]. The pathogenesis of JIA involves a varying combination of both autoimmune and environmental factors; the details of this process remain unclear. ...
... According to a multicenter study of 2030 individuals with JIA, the total prevalence of IgAD was 1 : 37, which is significantly higher than the population prevalence [13]. There are only a few publications concerning the relationship between IgA deficiency and JIA [6,14]. The case studies available consider the topic in terms of aberrations and do not address juvenile oligoarticular arthritis itself [15,16]. ...
Immunoglobulin A (IgA) deficiency is the most common primary immunodeficiency in humans, with incidence depending on ethnic background and the highest frequency in Caucasians. Selective IgA deficiency may have an asymptomatic course and constitute a random laboratory finding with no clinical manifestation. There is, however, a group of patients with increased incidence of recurrent upper respiratory tract infections, allergies, asthma, atopic dermatitis and other pathologies connected with IgA deficiency. This group of patients often needs broad-spectrum antibiotic therapy with maximum doses and extended time of treatment as there is no causal treatment for IgA deficiency. An association between IgA deficiency and autoimmune diseases, such as juvenile idiopathic arthritis, has been proved before. Nonetheless, the frequency of co-occurrence of these disorders in an individual as well as the way immunodeficiency may influence the course of juvenile idiopathic arthritis is still undefined, with limited literature on this topic. This article presents case reports of three pediatric patients with confirmed co-occurrence of IgA deficiency and oligoarticular juvenile idiopathic arthritis.
... Patients with an elevated ESR or unexplained anemia are more likely to have a recurrent disease and become an extended oligoarticular JIA. Differential diagnoses of oligoarticular JIA include the other types of JIA such as psoriatic arthritis, polyarticular JIA and ERA, Lyme disease, IBD, pigmented villonodular synovitis, other infectious, autoinflammatory, and autoimmune diseases, and malignancy, all of which may involve four or fewer joints at the onset [26,27]. ...
... include several diseases that may be self-limiting or chronic, including other forms of JIA such as psoriatic, systemic, enthesitis-related, reactive arthritis, earlyonset rheumatoid arthritis, systemic lupus erythematosus, systemic vasculitis, sarcoidosis, inflammatory bowel disease, epiphyseal dysplasia, and minocyclineinduced autoimmunity [27]. ...
Juvenile idiopathic arthritis (JIA) is the most common form of chronic synovial joint inflammation in children. It potentially leads to disability and psychosocial outcomes for children and their families. In the absence of appropriate treatment, this can lead to joint destruction and disability. Thus, early diagnosis and aggressive treatment are essential. With the presentation of new biologic DMARDs, based on understanding the disease pathophysiology and molecular pathogenesis, the course of the disease and its outcome have been changed profoundly. In this chapter, the early diagnosis, appropriate treatment, and outcomes approaches are described. These include the latest diagnosis and management options.
