[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"health-studies-list:{\"conditionNormalized\":\"stills-disease-adult-onset\",\"overallStatus\":[\"RECRUITING\",\"AVAILABLE\",\"NOT_YET_RECRUITING\"],\"orderBy\":\"LastUpdateSubmitDate:desc\",\"size\":25,\"offset\":0}":3,"health-study-condition:stills-disease-adult-onset":104},{"pageToken":4,"total":5,"offset":6,"count":5,"results":7},null,3,0,[8,47,75],{"id":9,"slug":4,"hasResults":10,"nctId":11,"briefTitle":12,"officialTitle":13,"acronym":4,"eligibilityCriteria":14,"healthyVolunteers":15,"sex":16,"minAge":17,"maxAge":18,"enrollmentInfo":19,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":23,"conditions":24,"keywords":29,"overallStatus":34,"whyStopped":4,"lastUpdateSubmitDate":35,"lastUpdatePostDateStruct":36,"startDateStruct":39,"completionDateStruct":41,"leadSponsor":43,"locationsCount":46},"100324933",false,"NCT03510442","Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still's Disease, and Related Conditions","Investigation of the Natural History, Genetics, and Pathophysiology of Systemic Juvenile Idiopathic Arthritis, Adult-Onset Still's Disease and Related Inflammatory Conditions","* INCLUSION CRITERIA:\n\nSubjects with known or suspected sJIA, AOSD or a similar inflammatory phenotype will provide informed consent and then be evaluated either in the outpatient or inpatient unit of the NIH Clinical Center. To be eligible for follow-up visits patients must meet the Inclusion Criteria, but not the Exclusion Criteria. Subjects determined to not have known or suspected sJIA or AOSD, or a related\n\ninflammatory phenotype, will not be followed.\n\nPatients with signs and symptoms of sJIA will be classified as outlined in #1, #2 and #3 below:\n\n1. Patients less than 16 years of age will be considered to have sJIA if they meet the ILAR criteria for sJIA.\n2. Patients 16 years of age and older will be considered to have sJIA if they have previously met ILAR criteria for sJIA.\n3. Family members of individuals included under items 1 and 2.\n4. Controls for clinical, cellular, molecular, and biochemical assays, and genetic evaluation will be enrolled. Individuals who undergo phlebotomy specifically to provide a control specimen will include both pediatric and adult patients and will not be pregnant.\n\nPatients with signs and symptoms of AOSD will be classified as outlined in #1, #2 and #3 below:\n\n1. Patients 16 years of age and older will be considered to have AOSD if they meet the Yamaguchi criteria for AOSD (including a negative ANA and RF).\n2. Patients may be considered to have a diagnosis of AOSD if they met criteria for diagnosis in the past but do not still have present evidence of disease.\n3. Family members of individuals included under items 1 and 2.\n4. Controls for clinical, cellular, molecular, and biochemical assays, and genetic evaluation will be enrolled. Individuals who undergo phlebotomy specifically to provide a control specimen will include both pediatric and adult patients and will not be pregnant.\n\nPatients with suspected sJIA, AOSD or a related inflammatory condition, as indicated by the presence of episodic fever and\u002For arthritis, may also be included.\n\nEXCLUSION CRITERIA:\n\n1. In adults, inability to provide informed consent and unavailability of a legally authorized representative to provide surrogate consent. In the case of minors, unavailability of a parent or guardian.\n2. Presence of any medical condition that would, in the opinion of the investigators, confuse the interpretation of the study.\n3. Unavailability, or inability to adhere with the schedule for follow-up visits.\n4. Pregnancy",true,"ALL","1 Day","100 Years",{"count":20,"type":21},2000,"ESTIMATED","OBSERVATIONAL","Background:\n\nInflammatory 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.\n\nObjective:\n\nTo study sJIA and AOSD in children and adults over time.\n\nEligibility:\n\nPeople with known or suspected sJIA, AOSD, or similar inflammatory condition\n\nDesign:\n\nParticipants will be screened with a phone call.\n\nParticipants 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:\n\n* Medical history\n* Physical exam\n* Musculoskeletal exam\n* Questions about overall health and quality of life, disease activity, functional status, and cognitive ability.\n\nParticipants may also have:\n\n* Pictures taken of their skin, joints, or spine\n* Blood, urine, and stool tests\n* Scans or X-rays of joints with arthritis\n* Chest X-ray\n* Heart tests\n* Skin biopsy. The skin will be numbed. The top layers of a small area will be scraped off.\n\nParticipants 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.\n\nParticipants who have a bone marrow biopsy may provide sample cells.\n\nParticipants may be seen by NIH specialists.\n\nMembers of the participant s family and healthy volunteers may give blood or saliva samples for genetic testing.\n\nParticipants may repeat some study tests every 6 months.",[25,26,27,28],"Still's Disease, Adult-Onset","Systemic Inflammation","Arthritis","Autoinflammatory Syndrome",[30,31,27,32,33],"Inflammation","Fever","Sequencing","Natural History","RECRUITING","2026-07-01",{"date":37,"type":38},"2026-07-02","ACTUAL",{"date":40,"type":38},"2018-05-21",{"date":42,"type":21},"2050-01-01",{"name":44,"class":45},"National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)","NIH",1,{"id":48,"slug":4,"hasResults":10,"nctId":49,"briefTitle":50,"officialTitle":51,"acronym":52,"eligibilityCriteria":53,"healthyVolunteers":10,"sex":16,"minAge":4,"maxAge":4,"enrollmentInfo":54,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":56,"conditions":57,"keywords":60,"overallStatus":34,"whyStopped":4,"lastUpdateSubmitDate":65,"lastUpdatePostDateStruct":66,"startDateStruct":68,"completionDateStruct":70,"leadSponsor":72,"locationsCount":46},"100510516","NCT05927454","Acostill ( RaDiCo Cohort) (RaDiCo Acostill)","Cohorte d'Adultes et d'Enfants Avec Maladie de Still","Acostill","Inclusion Criteria:\n\n* Aged over 16 (age\\> 16) meeting the diagnostic criteria of Yamaguchi or Fautrel criteria (appendix 5)\n* Aged 16 years or less (age ≤16 years) fulfilling the 2001 criteria for ILAR systemic form of juvenile idiopathic arthritis\n* Having signed a consent to participate in the cohort and in the collection of clinical and biological data; in accordance with the regulations, for patients who are minors or adults who are protected, the non-opposition of the legal representatives will be sought.\n* Affiliated to the \"Régime National d'Assurance Maladie\".\n\nExclusion Criteria:\n\n* Other cause of relapsing infectious fever (such as tuberculosis, toxoplasmosis, deep abscesses, viroses, sepsis) or tumor (such as lymphomas)\n* Other defined inflammatory rheumatism such as rheumatoid arthritis, psoriatic arthritis, spondyloarthropathies.\n* Autoimmune inflammatory disease (systemic lupus erythematosus), granulomatosis (sarcoidosis, Blau syndrome), vasculitis (Behçet's disease, nodular arteritis), polymyositis and dermatomyositis.\n* Well-defined auto-inflammatory syndromes with unambiguous mutations, such as familial Mediterranean fever, cryopyrinopathies, TRAPS, mevalonate kinase deficiency.\n* Known macrophage activation syndromes of genetic origin.\n* Patients unable to understand the information leaflet and sign the informed consent form\n* Patients not affiliated to the \"Régime National d'Assurance Maladie\"",{"count":55,"type":21},500,"Adult Onset Still Disease (AOSD) and Systemic onset Juvenile Idiopathic Arthritis (SoJIA) are two rare multifactorial diseases associated with systemic inflammation. These two forms AOSD and SoJIA are considered to be two facets of the same syndrome, combining four cardinal symptoms \\[hectic fever\\> 39 °, arthralgia or arthritis, skin rash, a leukocyte formula with more than 80% of neutrophils\\]; lymphadenopathy and splenomegaly may also be found. There is an important biological inflammatory syndrome with elevation of the reactive C protein, of serum ferritin with a dramatic drop in the glycosylated fraction. The incidence of the disease is low, around 0.1\u002F100,000 for adults and 0.6\u002F100,000 for children. Its prevalence is approximately 1 to 3\u002F100,000 and 3\u002F100,000 for children, so there are approximately 500 to 1,500 adults and 450 children affected in France. It is subdivided into pediatric and adult forms according to the age of onset before or after 16 years. The prognosis of the disease is functional and vital. Macrophage activation syndrome (SAM) is frequently associated with either the onset of the disease or the initiation of treatment or concomitantly with viral reactivation. The course over time has mainly been studied in children and is variable: regression, course by flare-ups with term regression and chronic joint development. In adults we can also observe these 3 evolutionary modes. However, differences seem to exist between AOSD and SoJIA.\n\nThe various clinical questions posed by this disease are as follows:\n\n* Why does it differentially affect two age groups of the population?\n* Why is the clinical expression heterogeneous with pure systemic or articular forms, the frequency of SAM, and rare organ damage?\n* Why is the evolution over time different with resolving monocyclic forms or polycyclic forms and sometimes chronic evolutions?\n\nThese differences could be explained by distinct underlying pathogenic mechanisms. But at present, the pathophysiology of this entity remains unknown, although several hypotheses can be formulated involving several pathophysiological pathways.\n\nThe pathogenesis of Still's disease has not yet been elucidated but there is a significant inflammatory reaction without the production of autoantibodies, which makes this disease a form of autoinflammatory syndrome with abnormalities of the innate immunity (activation of macrophages, strong elevations of pro-inflammatory cytokines: interleukins 1 and 18, possible abnormalities of inflammasomes and NK cells). The treatment is based on anti-inflammatory drugs, corticosteroids with the usefulness of methotrexate and anti-TNF in the event of significant joint damage. Interleukin 1 and 6 inhibitors have been shown to be effective in this disease. In adults and children, there are forms that are refractory to treatment, with a risk of AA amyloidosis for these patients.\n\nThe expected outcomes of this work are to improve knowledge of Still disease and patient management on the following aspects:\n\n* Comparison of pediatric and adult forms (which has never been done on a large number of patients),\n* Better understanding of the pathogenic mechanisms of the disease,\n* The identification of early diagnostic\u002Fprognostic markers,\n* The possibility of promoting the evaluation of new therapies to come thanks to the constitution of an active file of patients with a standardized follow-up.\n\nThe ACOSTILL study group is thus a unique collaboration of adult clinicians (rheumatologists and internists) and pediatricians, who have decided to unite their efforts to increase knowledge about the pathogenesis of Still disease in order to better understand the disease and improve care pathways. Many of them participated in the development of the national diagnostic and care protocol published in 2018.",[58,25,59],"Still Disease","Still Disease, Juvenile Onset",[58,61,62,63,64],"Rare disease","Diagnostic markers","Prognostic markers","New therapies","2026-02-10",{"date":67,"type":38},"2026-02-12",{"date":69,"type":38},"2017-07-11",{"date":71,"type":21},"2027-07",{"name":73,"class":74},"Institut National de la Santé Et de la Recherche Médicale, France","OTHER_GOV",{"id":76,"slug":4,"hasResults":10,"nctId":77,"briefTitle":78,"officialTitle":79,"acronym":80,"eligibilityCriteria":81,"healthyVolunteers":10,"sex":16,"minAge":4,"maxAge":82,"enrollmentInfo":83,"targetDuration":4,"studyType":22,"phases":4,"briefSummary":85,"conditions":86,"keywords":89,"overallStatus":34,"whyStopped":4,"lastUpdateSubmitDate":94,"lastUpdatePostDateStruct":95,"startDateStruct":97,"completionDateStruct":99,"leadSponsor":101,"locationsCount":46},"100220201","NCT02143986","Glycosylated Ferritin in Macrophagic Activation Syndromes","Glycosylated Ferritin in Differential Diagnosis of Still's Disease, Sepsis and Other Macrophagic Activation Syndromes.","FERRITGLY01","Inclusion Criteria:\n\n* Suspicion of sepsis, macrophagic activation syndrome, Still's disease or hyperferritinemia (malignant disease, hepatic cytolysis)\n\nExclusion Criteria:\n\n* Normal ferritin level","85 Years",{"count":84,"type":21},60,"In healthy subjects, from 50 to 80 % of the serum ferritin is glycosylated \\[1, 2\\] . A decrease in the percentage of ferritin glycosylation can be observed in inflammatory diseases, malignancies, infections, or liver disease but is rarely less than 20% \\[3 , 4\\] . Percentage of glycosylated ferritin below 20% have been described in patients with adult Still's disease and haemophagocytosis lymphohistiocytic syndromes (HLH).\n\nThe glycosylated ferritin has been included in the diagnostic criteria for Still's disease in adults. A cut-off of less than 20 % has a sensitivity and specificity of 72 and 69 % respectively , and 35 and 94 % when combined with a total ferritin level greater than 5 times normal value. This parameter was also suggested to be a more specific marker to confirm a diagnosis of HLH than a high ferritin level ( \\> 500μg \u002F L). However, several limitations of this parameter were highlighted, some conditions making its interpretation difficult : particularly in cases of major hepatic cytolysis and severe sepsis (miliary tuberculosis, lymphoma and disease Adult Still).\n\nIt is not always possible to distinguish severe sepsis, HLH syndrome and Still's disease.\n\nA fine analysis of various glycoforms components of ferritin could be used to distinguish different subgroups of patients. Few data are available on the mechanism of secretion and glycosylation of ferritin, but the investigators assume that the glycosylation patterns of ferritin may vary between different disease states and reflect distinct underlying pathophysiological mechanisms.",[25,87,88],"Sepsis","Macrophagic Activation Syndrome",[90,91,92,93],"sepsis","Macrophagic activation syndrome","Ferritin","Still's disease","2025-10-01",{"date":96,"type":38},"2025-10-06",{"date":98,"type":4},"2014-05",{"date":100,"type":21},"2030-12",{"name":102,"class":103},"Brugmann University Hospital","OTHER",""]