Do infections trigger juvenile idiopathic arthritis?

Juvenile idiopathic arthritis (JIA) is a disease that was prominent with increased inflammation response in immune system, appeared mostly with peripheral arthritis and endogenous and exogenous antigens play a role in the pathogenesis of disease. Two major reasons were thinking to be considerably important. First of them is immunological predisposition and the second one is environmental factors. Infections are considered to be the most important between environmental factors but also stress and trauma are also important in the etiology of the disease. However, the relation between JIA and infections is not clearly defined but the relation between adult chronic arthritis and infections was well-defined. A total of 70 patients, 26 with primer JIA, 20 with recurrent JIA, 24 healthy control were included in this study. Mycoplasma pneumoniae, Chlamydophila pneumoniae and C. Jejuni were detected in 4, 1 and 1 of 10 (38.46%) patients with primer JIA, respectively. Salmonella enteritidis, EBV, M. pneumoniae, C. jejuni and Borrelia burgdorferi were detected in 1, 2, 2, 2, and 1 of the 8(40%) patients with recurrent JIA, respectively. S. enteritidis were isolated in feces culture and also identified by agglutination method. Infection was detected in total 18 (39.13%) of patient groups. C. pneumoniae and C. jejuni were detected in 1 and 1 of 2(8.33) healthy control groups, respectively. Throat culture positivity was not detected in any of the patient and healthy control groups. In conclusion, etiopathogenesis of JIA is not clearly understood and suggested that various factors can trigger the disease and it is the most common rheumatoid disease of childhood. However, there are some studies focusing especially on one infectious agent but this is the first study including such a big range of infectious agents in the literature for the microorganisms that can be suggested to have a role in the etiopathogenesis of JIA. We have a conclusion in the light of our results and suggest that some microorganisms can trigger and increase the intensity of clinical situation according to the case. When we evaluate the primer and recurrent JIA groups; M. pneumoniae and C. jejuni come forward and seen common in JIA cases. We also suggest that the pre-diagnosis of microorganisms, which can play a role as primarily or by intervening in the etiopathogenesis of JIA and adding specific antimicrobial therapy to the standard JIA therapy, it is possible to perform new, extended, especially molecular based serial case studies.     

Primary headaches in pediatric patients with chronic rheumatic disease

Objectives: To assess the presence, prevalence and clinical characteristics of primary headaches in pediatric patients with chronic rheumatic diseases such as juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF), and to analyze the common pathophysiological mechanisms. Study design: In this noncontrolled, cross-sectional study, a semi-structured 53 item headache questionnaire was administered to subjects with FMF and JIA, and interviewed a total sample size of 601 patients younger than16 years of age. The questionnaires were then analyzed according to the International Headache Society’s diagnostic criteria. Results: Children with FMF (n = 378) and JIA (n = 223) were studied. Each group was then divided into two subgroups according to whether the subjects reported headache or not. 29.5% of subjects with FMF reported having migraine, 37.6% probable migraine and 32.9% tension type headache (TTH). In JIA group 28.2% were diagnosed with migraine; 41.2% with probable migraine and 30.6% with TTH. No significant difference was found between all subjects with (n = 258) and without (n = 343) headache for variables such as living in a crowded family (p = 0.95), being the first child in the family (p = 0.63), academic achievement of the child (p = 0.63), high education level (higher than high school) of the mother (p = 0.52) and father (p = 0.46). The presence of systemic disease was reported not to be effecting the daily life at the time of evaluation by 90.2% of the children with headache and 91.0% of the children without headache (p = 0.94). 81.4% of the children reported their headaches were not aggravating with the exacerbation periods of their systemic disease. Family history of hypertension was reported higher by the subjects with headache (13.5% with headache and 4.0% without headache p = 0.001). Diabetes mellitus was also reported higher (5.8% with headache; 0.5% without headache; p = 0.006). Family history of headache was reported in 28.2% of the patients with headache whereas it was 17.4% of the patients without headache (p < 0.001). Family history of headache was reported in 28.2% of the FMF subjects with headache whereas it was 17.4% of the patients without headache (p < 0.001). For JIA patients a positive family history for headache was obtained in 25.9% of children with headache notably in migraineurs (81.8%). Conclusion: Patients with JIA and FMF should be asked specifically about accompanying primary headaches particularly migraine headaches as they may be additional disabilities for these patients.     

Glucose intolerance: is it a risk factor for cardiovascular disease in children with chronic kidney disease?

A total of 66 children and adolescents with chronic kidney disease (CKD) (20 pre-dialysis patients and 46 chronic dialysis patients) were evaluated to address the prevalence of abnormalities in glucose and insulin metabolism and their association with cardiovascular disease. Glucose intolerance was assessed using an oral glucose tolerance test; insulin resistance was estimated by the homeostasis model assessment of insulin resistance (HOMA-IR). Carotid artery intima-media thickness (IMT) and left ventricular hypertrophy (LVH) were examined as early markers of cardiovascular disease. Thirty-four patients (7 pre-dialysis, 27 dialysis) exhibited an abnormal glucose tolerance; however, ten patients (7 pre-dialysis, 3 dialysis) were insulin-resistant. Height-specific standard deviation scores of carotid artery IMT were above normal in three of the pre-dialysis patients (15%) and in 34 of the dialysis patients (74%). LVH was present in seven pre-dialysis (35%) and 34 dialysis patients (74%). In addition, two of the pre-dialysis patients (10%) and 18 of the dialysis patients (39%) had severe LVH. The prevalence of both increased carotid artery IMT and LVH were similar in patients with or without glucose intolerance in both groups, but severe LVH was more prevalent in pre-dialysis patients with glucose intolerance (p = 0.042). The multivariate analyses showed that neither carotid artery IMT nor LVM index was predicted by serum glucose levels or HOMA-IR. In conclusion, children with CKD are at a high risk of glucose intolerance and also have a greater risk of subclinical cardiovascular disease (CVD). However, the presence of glucose intolerance does not appear to be an independent risk factor for increased carotid artery IMT or LVH.     

Cross-cultural adaptation, reliability, and validity of the Turkish version of PedsQL 3.0 Arthritis Module: a quality-of-life measure for patients with juvenile idiopathic arthritis in Turkey

Purpose

The aim of this study was to describe the cultural adaptation, validity, and reliability of a Turkish version of the pediatric quality-of-life inventory (PedsQL) 3.0 Arthritis Module in a population with juvenile idiopathic arthritis (JIA).

Methods

A total of 169 patients with JIA and their parents were enrolled in the study. The Turkish version of the childhood health assessment questionnaire (CHAQ) was used to evaluate the validity of related domains in the PedsQL 3.0 Arthritis Module. Both the PedsQL 3.0 Arthritis Module and CHAQ were filled out by children over 8 years of age and by the parents of children 2–7 years of age.

Results

Internal reliability was poor to excellent (Cronbach’s alpha coefficients 0.56–0.84 for self-reporting and 0.63–0.82 for parent reporting), and interobserver reliability varied from good to excellent (intraclass correlation coefficient (ICC) 0.79–0.91 for self-reporting and 0.80–0.88 for parent reporting) for the total scores of the PedsQL 3.0 Arthritis Module. Parent–child concordance for all scores was moderate to excellent (ICC 0.42–0.92). The PedsQL 3.0 Arthritis Module and CHAQ were highly positively correlated, with coefficients from 0.21 to 0.76, indicating concurrent validity.

Conclusions

We demonstrated the reliability and validity of quality-of-life measurement using the Turkish version of the PedsQL 3.0 Arthritis Module in our sociocultural context. The PedsQL 3.0 Arthritis Module can be utilized as a tool for the evaluation of quality of life in patients with JIA aged 2–18 years.     

The relationship between physical activity level,anxiety, depression,and functional ability in children and adolescents with juvenile idiopathic arthritis

The aim of this study was to assess the relationships between physical activity level and anxiety, depression, and functional ability in children and adolescents with juvenile idiopathic arthritis (JIA). Cross-sectional study design including patients with JIA aged between 8 and 17 years and healthy controls was used. Sociodemographic data and clinical features were assessed. Physical activity level and energy expenditure were assessed with a 1-day activity diary. Anxiety was screened by The Screen for Child Anxiety Related Emotional Disorders (SCARED) questionnaire. Depressive symptoms were assessed by the Children’s Depression Inventory (CDI). Functional ability was assessed with the Childhood Health Assessment Questionnaire (CHAQ). Pain and overall well-being were measured using a visual analog scale (VAS). Fifty-two patients and 48 controls were included with a mean age of 12.13 ± 2.92 and 11.27 ± 1.59 years, respectively. The mean disease duration was 64 months. The JIA group had significantly less time in physical activity (p = 0.000), decrease in energy expenditure (p = 0.04), and higher CHAQ scores (p = 0.000) compared with the control group. In the JIA group, significant relationships were found between the number of active joint and disease duration (r = 0.44, p = 0.000) and VAS pain (r = 0.30, p = 0.02), between SCARED and CDI (r = 0.54, p = 0.000). Significant relationships were found between VAS overall well-being and CDI (r = 0.29, p = 0.03), CHAQ (r = 0.37, p = 0.000), and VAS pain (r = 0.41, p = 0.000). Correlation between CHAQ and CDI (r = 0.34, p = 0.01) was significant. The result of our study suggested that only depression was related to anxiety, functional ability, and well-being in children and adolescents with JIA.     

NLRP3 gene variants and serum NLRP3 levels in periodic fever,aphthous stomatitis,pharyngitis, and adenitis (PFAPA) syndrome

Clinical Rheumatology - Although most of the autoinfammatory disorders have a confirmed genetic cause, periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome still...     

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease in childhood, which represents a nonhomogeneous group of disorders that share the clinical manifestation of arthritis lasting at least 6 wk under the age of 16. The exact diagnosis requires exclusion of other diseases that cause arthritis. The exact etiopathogenesis of JIA is still unknown. The interactions between genetic factors, environmental exposures and immune mechanisms are thought to contribute to pathogenesis of the disease. The “International League Against Rheumatism” classification divides JIA into 7 subtypes: oligoarticular JIA, rheumatoid factor (RF) positive polyarticular JIA, RF negative polyarticular JIA, systemic-onset JIA, enthesitis-related arthritis, juvenile psoriatic arthritis and undifferentiated JIA. Each subgroup of JIA is characterized by a different mode of presentation, disease course and outcome. The improvements in treatment of JIA in the last 2 decades, such as the early introduction of intraarticular corticosteroids, methotrexate and biologic agents, have dramatically upgraded the prognosis of the disease. If untreated, JIA may cause devastating results, such as disability from joint destruction, growth retardation, blindness from chronic iridocyclitis, and even multiple organ failure and death in systemic-onset JIA. The aim of treatment is the induction of remission and control the disease activity to minimize the pain and loss of function, and to maximize quality of life. JIA is a disease having a chronic course, which involves active and inactive cycles over the course of years. Recent studies showed that nearly half of the patients with JIA enter adulthood with their ongoing active disease. This review elucidates how recent advances have impacted diagnosis, pathogenesis and current treatment.