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Adult-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:
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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| children and adult diagnosed with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital | children and adult with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| children and adult with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital | Other | Comparison of Adult-Onset Still's disease phenotypes |
|
| Measure | Description | Time Frame |
|---|---|---|
| validation of classification criteria | number of participants with clinical and biological abnormalities, as assessed in the Yamaguchi and Fautrel criteria | at inclusion |
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Inclusion Criteria:
Exclusion Criteria:
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children and adult diagnosed with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Yvan Jamilloux, Dr | Contact | +33426732636 | yvan.jamilloux@chu-lyon.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital de la Croix Rousse | Recruiting | Lyon | Auvergne-Rhône-Alpes | 69004 | France |
The individual participant data will not be shared
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| Centre Hospitalier de Valence | Recruiting | Valence | Drome | 26953 | France |
|
| Centre Hospitalier d'Annecy | Recruiting | Annecy | 74370 | France |
|
| Centre Hospitalier de Bourg en Bresse | Recruiting | Bourg-en-Bresse | 01012 | France |
|
| Centre Hospitalier Bourgoin Jallieu | Recruiting | Bourgoin | 38300 | France |
|
| Centre Hospitalier de Chambéry | Recruiting | Chambéry | 73000 | France |
|
| Centre Hospitalier de Châlon sur Saône | Recruiting | Châlon Sur Saône | 71321 | France |
|
| Centre Hospitalier Alpes-Léman (CHAL) | Recruiting | Contamine-sur-Arve | 74130 | France |
|
| Centre Hospitalier du puy en velay | Recruiting | Le Puy-en-Velay | 43000 | France |
|
| Centre Hospitalier de Limoges | Recruiting | Limoges | 87000 | France |
|
| Hopital Saint Luc Saint Joseph | Recruiting | Lyon | 69007 | France |
|
| Hospices Civils de Lyon - Edouard Herriot | Recruiting | Lyon | 69008 | France |
|
| Centre Hospitalier de Montélimar | Recruiting | Montélimar | 26200 | France |
|
| Centre Hospitalier Vienne | Recruiting | Vienne | 38200 | France |
|
| Centre Hospitalier de Villefranche | Recruiting | Villefranche-sur-Saône | 69400 | France |
|
| Médipôle de Villeurbanne | Recruiting | Villeurbanne | 69100 | France |
|
| ID | Term |
|---|---|
| D001171 | Arthritis, Juvenile |
| ID | Term |
|---|---|
| D001168 | Arthritis |
| D007592 | Joint Diseases |
| D009140 | Musculoskeletal Diseases |
| D012216 | Rheumatic Diseases |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D001327 | Autoimmune Diseases |
| D007154 | Immune System Diseases |
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