ITMO Santé Publique
ITMO Santé Publique
Date de modification : 21/03/2024 | Version : 1 | ID : 74149
| Général | |
| Identification | |
| Nom détaillé | Adult and pediatric cohort with Still's disease (RaDiCo-ACOSTILL) |
| Sigle ou acronyme | RaDiCo-ACOSTILL |
| Numéro d'enregistrement (ID-RCB ou EUDRACT, CNIL, CPP, etc.) | N° CCTIRS 16-088bis / N° CPP 14128 ND / N° MESR DC-2015-2479 / MR-001 |
| Thématiques générales | |
| Domaine médical |
Internal medicine Pediatrics Rare diseases Rheumatology |
| Etude en lien avec la Covid-19 |
No |
| Pathologie, précisions | Adult Still's disease (AOSD) and systemic onset juvenile idiopathic arthritis (SoJIA) represent two rare multifactorial diseases associated with systemic inflammation. These two forms, AOSD and SoJIA, are considered two facets of the same syndrome, combining four cardinal symptoms [high fever > 39°C, arthralgia or arthritis, skin rash, leukocyte formula with more than 80% neutrophil polymorphonuclear cells]; lymphadenopathy and splenomegaly may also be present; there is significant biological inflammatory syndrome with elevated C-reactive protein, serum ferritin with a dramatic decrease in glycosylated fraction. The incidence of the disease is low, around 0.1 / 100,000 for adults and 0.6 / 100,000 for children. Its prevalence is approximately 1 to 3/100,000 and 3/100,000 for children, so there are approximately 500 to 1500 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 (MAS) is frequently associated, either at the onset of the disease, at the initiation of treatment, or concomitant with viral reactivation. The evolution over time has mainly been studied in children and is variable: regression, episodic progression with regression over time, and chronic joint evolution. In adults, these three evolutionary modes can also be observed. However, differences seem to exist between AOSD and SoJIA. |
| Responsable(s) scientifique(s) | |
| Nom du responsable | Georgin-Lavialle |
| Prénom | Sophie |
| Adresse | Hôpital TENON, 4 rue de la Chine, 75020 PARIS. |
| Téléphone | 0033(0) 1 56 01 60 77 |
| sophie.georgin-lavialle@aphp.fr | |
| Laboratoire | UMRS 938 |
| Organisme | National Institute of Health and Medical Research (Inserm) |
| Nom du responsable | Fautrel |
| Prénom | Bruno |
| Adresse |
Groupe Hospitalier Universitaire Pitié-Salpêtrière 83 boulevard de l'Hôpital, 75651 Paris cedex 13, France |
| Téléphone | +33(0) 1 421 77801 |
| bruno.fautrel@aphp.fr | |
| Laboratoire | UMR S 1136 |
| Organisme | National Institute of Health and Medical Research (Inserm) |
| Collaborations | |
| Financements | |
| Financements |
Public |
| Précisions | The RaDiCRaDiCo-ACOSTILL cohort initially received funding from the state managed by the National Research Agency (ANR) as part of the "Investissements d'Avenir" cohorts program (PIA). |
| Gouvernance de la base de données | |
| Organisation(s) responsable(s) ou promoteur | National Institute of Health and Medical Research (Inserm) |
| Statut de l’organisation |
Secteur Public |
| Existence de comités scientifique ou de pilotage |
Yes |
| Labellisations et évaluations de la base de données | Security audit certification of the database. Data management and continuous quality control of data. |
| Contact(s) supplémentaire(s) | |
| Caractéristiques | |
| Type de base de données | |
| Type de base de données |
Morbidity registers |
| Base de données issues d'enquêtes, précisions |
Cohort study |
| Origine du recrutement des participants |
A selection of health institutions and services |
| Critère de sélection des participants |
Another treatment or procedure |
| Le recrutement dans la base de données s'effectue dans le cadre d'une étude interventionnelle |
No |
| Informations complémentaires concernant la constitution de l'échantillon | All pediatric and adult patients already diagnosed and followed (prevalent patients) or newly diagnosed (incident patients) in one of the French Reference Centers for Rare Diseases or Rare Disease Competence Centers will be invited to participate in the study. In order to document the improvement in patient management, morbidity, and mortality through the implementation of the Still's Disease National Diagnostic and Care Guidelines (PNDS Still), deceased patients may be included in the cohort. The objective is to recruit a minimum of 200 adult patients and 300 pediatric patients to ensure that the study has sufficient statistical power. |
| Objectif de la base de données | |
| Objectif principal | The main objective is to describe the natural history of the disease in adult and pediatric populations. |
| Critères d'inclusion |
The inclusion criteria for the RaDiCo-ACOSTILL cohort are as follows:
- Patients aged over 16 years (age >16 years) meeting the Yamaguchi diagnostic criteria or Fautrel criteria. - Patients aged 16 years or younger (age ≤16 years) fulfilling the 2001 criteria for systemic onset juvenile idiopathic arthritis according to the ILAR classification. - Having signed consent to participate in the cohort and for the collection of clinical and biological data; in accordance with regulations, for deceased minors or adults under legal protection, non-opposition from legal representatives will be sought. - Affiliated with the social security system. The exclusion criteria are: - Other causes of recurrent infectious fever (such as tuberculosis, toxoplasmosis, deep abscesses, viral infections, sepsis) or tumor-related fever (such as lymphomas). - Other defined inflammatory rheumatic diseases such as rheumatoid arthritis, psoriatic arthritis, spondyloarthropathies. - Autoimmune inflammatory diseases (systemic lupus erythematosus), granulomatosis (sarcoidosis, Blau syndrome), vasculitis (Behçet's disease, polyarteritis nodosa), polymyositis, and dermatomyositis. - Well-defined autoinflammatory syndromes with unambiguous mutations, such as familial Mediterranean fever, cryopyrinopathies, TRAPS, mevalonate kinase deficiency. - Known genetic macrophage activation syndromes. - Patients unable to understand the information leaflet and sign the informed consent form. - Patients not affiliated with the social security system. |
| Type de population | |
| Age |
Newborns (birth to 28 days) Infant (28 days to 2 years) Early childhood (2 to 5 years) Childhood (6 to 13 years) Adolescence (13 to 18 years) Adulthood (19 to 24 years) Adulthood (25 to 44 years) Adulthood (45 to 64 years) Elderly (65 to 79 years) Great age (80 years and more) |
| Population concernée |
Sick population |
| Pathologie | M05-M14 - Inflammatory polyarthropathies |
| Sexe |
Male Woman |
| Champ géographique |
National |
| Collecte | |
| Dates | |
| Année du premier recueil | 2017 |
| Année du dernier recueil | 2027 |
| Taille de la base de données | |
| Taille de la base de données (en nombre d'individus) |
< 500 individuals |
| Détail du nombre d'individus | 422 |
| Données | |
| Activité de la base |
Current data collection |
| Type de données recueillies |
Clinical data Declarative data Paraclinical data Biological data |
| Données cliniques, précisions |
Direct physical measures Medical registration |
| Détail des données cliniques recueillies | The main variables collected are: demographics, family and medical history, socio-economic data, clinical and biological data, clinical signs, symptoms, specific disease treatments, and quality of life self-assessment questionnaires. |
| Données déclaratives, précisions |
Paper self-questionnaire Internet self-questionnaire |
| Détail des données déclaratives recueillies | HAQ/CHAQ child and parent / SF 36 - SF10 (adult - child) / Psychological impact (Hamilton, adult) / Impact on work productivity and activity (WPAI, adult) / Perceived impact (PASS MCII, adult) |
| Données paracliniques, précisions | Imaging data (standard radiographs of painful joints when performed) |
| Données biologiques, précisions | Biochemical, hematological, and immunological data |
| Existence d’une biothèque |
Yes |
| Contenu de la biothèque |
Whole blood Plasma DNA |
| Détail des éléments conservés | This study includes a collection of biological samples conducted as part of research for future studies (DNA, RNA, plasma, peripheral blood mononuclear cells). |
| Paramètres de santé étudiés |
Health event/morbidity Health event/mortality Health care consumption and services Quality of life/health perception Others |
| Consommation de soins, précisions |
Hospitalization Medical/paramedical consultation Medicines consumption |
| Modalités | |
| Mode de recueil des données | eCRF in secure web access, secure cloud and HADS hosting |
| Nomenclatures employées | Drug Dictionary (DCIs) |
| Procédures qualité utilisées | Data Management Plan and Data Validation Plan. Continuous data management (automatic control rules and query system) |
| Suivi des participants |
Yes |
| Modalités de suivi des participants |
Monitoring by convocation of the participant Monitoring by contact with the referring doctor |
| Appariement avec des sources administratives |
No |
| Valorisation et accès | |
| Valorisation et accès | |
| Accès | |
| Existence d’un document qui répertorie les variables et les modalités de codage |
Yes |
| Charte d'accès aux données (convention de mise à disposition, format de données et délais de mise à disposition) | Requests for access to RaDiCo-ACOSTILL data (aggregated or individual) will be reviewed by the Scientific Committee following the submission of a summary of a Specific Research Project, as defined in the Access Charter. Requests should be sent to the following address: acostill@radico.fr |
| Accès aux données agrégées |
Access on specific project only |
| Accès aux données individuelles |
Access on specific project only |
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