Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset...
Still's DiseaseAdult-Onset3 moreBackground: Inflammatory conditions can cause symptoms like fevers, arthritis, and rash. Systemic juvenile idiopathic arthritis (sJIA) is one of these conditions. So is adult-onset Still s disease (AOSD). Their causes are unknown. Researchers want to learn more about these conditions. This includes genetic changes and environmental factors. Objective: To study sJIA and AOSD in children and adults over time. Eligibility: People with known or suspected sJIA, AOSD, or similar inflammatory condition Design: Participants will be screened with a phone call. Participants will have 1 visit. It may be outpatient or they may be admitted to the clinic. The visit may last up to 5 days. Participants will have: Medical history Physical exam Musculoskeletal exam Questions about overall health and quality of life, disease activity, functional status, and cognitive ability. Participants may also have: Pictures taken of their skin, joints, or spine Blood, urine, and stool tests Scans or X-rays of joints with arthritis Chest X-ray Heart tests Skin biopsy. The skin will be numbed. The top layers of a small area will be scraped off. Participants who have a joint aspiration may provide a fluid sample. The joint will be prepared, then fluid is removed by needle. A corticosteroid may be injected. Participants who have a bone marrow biopsy may provide sample cells. Participants may be seen by NIH specialists. Members of the participant s family and healthy volunteers may give blood or saliva samples for genetic testing. Participants may repeat some study tests every 6 months.
Development of a Therapeutic Endpoint in Pediatric Rheumatologic Conditions
Juvenile Idiopathic ArthritisSystemic Lupus Erythematosus1 moreThe overarching goal of this study is the development of a physiologic endpoint of pain and treatment effect in three distinct rheumatology populations. This would enable objective assessment of pain and treatment in these populations and enable a much more precise approach to treatment. Such an endpoint stands to significantly improve outcomes in these patients by eliminating the need for a trial-and-error approach to treatment. This is a single site observational study that aims to collect initial pilot data in three distinct patient groups. As this is observational, there is no randomization or blinding in the study. Patients will be followed for a period of one year after enrollment. Baseline measurements will be taken at the time of enrollment, and at each subsequent standard of care clinic visit as feasible, for a period of one year. As this is an observational study, there will be no change to the treatment for any patient due to research activities. The primary objective of this study is the characterization of the nociceptive index in three pediatric rheumatology populations. The secondary objective is the characterization of the nociceptive index in these populations in response to standard of care interventions. This is necessary to demonstrate the ability of this approach to serve as an endpoint of treatment effect.
Turkish Validity and Reliability of Pain Catastrophizing Scale-Child (PCS-C)
Chronic PainJuvenile Idiopathic ArthritisPerception of chronic pain and related disability; occurs through the interaction of physiological and psychological processes. Pain catastrophizing is a cognitive attribution style characterized by a negative mindset and magnification of pain. Catastrophizing in children has been associated with poor functioning and higher levels of pain. Catastrophizing during the transition to adulthood is defined as an important predictor of persistent pain and central sensitivity. The number of scales assessing pain and attitudes related to pain in children is quite low. In recent years, with the adaptation of the assessment scales used for adults to children or the development of new scales, the assessment of pain and pain-related attitudes in children has begun to be provided. Pain catastrophizing scale (PCS), in 1995 Sullivan et al. for the purpose of comprehensive assessment in adults experiencing pain-related disasters. In 2003, Crombez et al. showed the validity and reliability of the scale in school-age children. For predictive validity, the scale was administered to children with chronic pain, and it was reported to predict the severity of pain and pain-related disability. Pain Catastrophizing Scale-Child (PCS-C) scale; It is a self-report measure adapted from the Adult Pain Catastrophizing Scale used to assess negative thinking associated with pain. It contains 13 items rated on a 5-point scale ranging from 0 = "not at all true" to 4 = "very true". Substances; rumination (4 items, e.g. "When [my child] has pain, I can't get him out of my mind"), magnification (3 items, e.g. the child has pain], thinking about other painful events"), and helplessness (6 items, e.g. "[My child's]] When I have pain, I feel that I cannot continue"). Items are aggregated across subscales to obtain a total score ranging from 0 to 52; higher scores are related to higher catastrophizing attitude. The pain catastrophizing scale was originally developed in German and later validity and reliability studies were conducted in different languages. The aim of this study is to question the Turkish validity and reliability of the "Pain Catastrophizing Scale-Child (PCS-C)" scale.
Imaging Based Uveitis Screening for Children With Juvenile Idiopathic Arthritis
UveitisAnterior1 moreThis study seeks to describe, for children undergoing uveitis surveillance following a new diagnosis of juvenile idiopathic arthritis, the feasibility metrics of undertaking a randomised comparative study of routine slit lamp examination (SLE) versus imaging based (anterior segment optical coherence tomography, OCT) surveillance in order to inform the development of a larger multi-centre trial.
Juvenile Inflammatory Rheumatism (JIR) Cohorte
ArthritisJuvenile RheumatoidThe investigators created a cohort of patients with juvenile inflammatory rheumatisms with the purpose to follow them prospectively, and investigate the tolerance and efficacy of immunosuppresive and biological agents.
Angiogenic Biomarkers in Juvenile Idiopathic Arthritis
Juvenile Idiopathic Arthritis (JIA)The aim of the study is to determine whether serum inflammatory angiogenic markers (eg, semaphorins, CCN1) predict severity of juvenile idiopathic arthritis defined by structural progression and/or therapeutic escalation.
Scoring System of Structural Damage for the Hip in Juvenile Idiopathic Arthritis
Juvenile Idiopathic Arthritis HipJuvenile idiopathic arthritis (JIA) is a chronic inflammatory disease in children affecting mobility and physical function. The hip involvement represents a frequent complication in JIA patients. The assessment of hip damage becomes a mandatory step in disease monitoring. However, radiological scoring was not standardized. This study aimed to compare the two scoring systems previously proposed, examine their repeatability and their intra and inter agreement.
Auvergne-Rhône-Alpes-Limousin Research Database for Still's Diseases in Children and Adults
Still DiseaseAdult-Onset Still's disease is a polygenic autoinflammatory disease of unknown etiology. The autoinflammatory character individualizes it from autoimmune autoantibody diseases. Clinically, it results in the classic triad associating hectic fever, evanescent rash and arthritis. Although it is benign in the vast majority of cases, life-threatening complications can occur. By definition, the disease affects adults over 16 years of age, however most experts now agree that the adult form and the pediatric form belong to a pathological continuum: Still's disease. In the absence of a specific biomarker, the diagnosis is still based on clinical and biological criteria, after the exclusion of differential diagnoses. Classically, three evolutionary profiles of Adult-Onset Still's disease are individualized, depending on the evolution of symptoms over time: a monocyclic systemic form (30% of cases) characterized by clear systemic symptoms and in the foreground compared to the articular signs. This form evolves over several weeks to several months (on average 9 months), without exceeding a year. By definition, there is no recurrence; a polycyclic systemic form (30% of cases) defined by the occurrence of at least two systemic or joint episodes, separated by clinical remission intervals greater than two months, or even several years. The symptoms of relapses are not always the same as the initial symptoms. The number and severity of relapses is unpredictable and varies widely from patient to patient, but symptoms tend to become less severe over time. a chronic form, with predominant joint involvement (40%), resembling seronegative rheumatoid arthritis. Systemic signs are present during the first outbreaks of the disease. Subsequently, rheumatoid arthritis evolves on its own and one can see joint destruction or conversely ankylosing developments such as the classic bilateral, non-erosive fusing carpitis. There are reasons to believe that the evolving profile of patients has changed since the emergence and generalization of biotherapies. Furthermore, no prognostic factor for the progression of Adult-Onset Still's disease has been found so far. The differences between pediatric and adult forms need to be confirmed and becoming pediatric forms in adulthood is poorly described. The objective of this study is to set up a regional research database (Auvergne-Rhône-Alpes-Limousin) in order to describe the characteristics, treatment and evolution of patients with Still's disease.
Access to Pediatric Rheumatology Centers for JIA Patients: Factors Associated With Time to Access...
Juvenile Idiopathic ArthritisJIA patients, their parents, and the health care professionals experience the complexity of the initial care pathway. The aim of the study is to explore the referral pathway to access pediatric rheumatology centers for JIA patients. The exploration will aim to identify the barriers and facilitators of referral, based on the conceptual framework of the health literacy. The investigators will conduct a qualitative study using semi-structured interviews. The perspectives of parents/children/health care professionals will be crossed to enrich the data.
Host-microbiota-environment Interactions
Juvenile Idiopathic ArthritisDiabetes type11 moreTwo types of inflammatory and autoimmune diseases (excluding monogenic diseases) can be distinguished in children: those similar to adult diseases but with an early onset (type 1 diabetes, inflammatory diseases of the gastrointestinal tract, rheumatoid arthritis with anti-CCP antibodies) and those specific to children that are not described in adults (early-onset juvenile idiopathic arthritis with anti-nuclear and anterior uveitis). The familial and nosological aggregations suggest that these diseases are probably polygenically determined, and result from interactions with the environment. In a singular way, the incidence of "adult" diseases is increasing while the age of onset is getting earlier; conversely, there is no increase in early-onset juvenile idiopathic arthritis. On the other hand, the influence of early events that may alter the microbiotic environment is different for different diseases: whereas cesarean section (or early antibiotic therapy) has been shown to increase the risk of JIA and T1DM, it does not seem to change the risk of IBD. We hypothesize that environmental factors, particularly those related to diet and bacterial and fungal digestive microbiota - are different between these disease categories.