Auvergne-Rhône-Alpes-Limousin Research Database for Still's Diseases in Children and Adults

NCT05055882 | Recruiting

• 1 other identifier: CRC_GHN_2021_003
• Study Type: observational
• Target: 400
• Locations: 1 country
• Sites: 16

Brief Summary

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:

• 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.

Conditions

Still Disease

Outcome Measures

Primary Outcomes (1)

• validation of classification criteria

  number of participants with clinical and biological abnormalities, as assessed in the Yamaguchi and Fautrel criteria

  at inclusion

Study Arms (1)

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

Other: children and adult with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital

Interventions

children and adult with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital OTHER

Comparison of Adult-Onset Still's disease phenotypes

children and adult diagnosed with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital

Eligibility Criteria

• Age: 3 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

children and adult diagnosed with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital

You may qualify if:

• children and adult diagnosed with Still's disease in Auvergne-Rhône-Alpes-Limousin hospital

You may not qualify if:

• Not applicable

Sponsors & Collaborators

• Hospices Civils de Lyon: lead

Study Sites (16)

Hôpital de la Croix Rousse

Lyon, Auvergne-Rhône-Alpes, 69004, France

RECRUITING

Centre Hospitalier de Valence

Valence, Drome, 26953, France

RECRUITING

Centre Hospitalier d'Annecy

Annecy, 74370, France

RECRUITING

Centre Hospitalier de Bourg en Bresse

Bourg-en-Bresse, 01012, France

RECRUITING

Centre Hospitalier Bourgoin Jallieu

Bourgoin, 38300, France

RECRUITING

Centre Hospitalier de Chambéry

Chambéry, 73000, France

RECRUITING

Centre Hospitalier de Châlon sur Saône

Châlon Sur Saône, 71321, France

RECRUITING

Centre Hospitalier Alpes-Léman (CHAL)

Contamine-sur-Arve, 74130, France

RECRUITING

Centre Hospitalier du puy en velay

Le Puy-en-Velay, 43000, France

RECRUITING

Centre Hospitalier de Limoges

Limoges, 87000, France

RECRUITING

Hopital Saint Luc Saint Joseph

Lyon, 69007, France

RECRUITING

Hospices Civils de Lyon - Edouard Herriot

Lyon, 69008, France

RECRUITING

Centre Hospitalier de Montélimar

Montélimar, 26200, France

RECRUITING

Centre Hospitalier Vienne

Vienne, 38200, France

RECRUITING

Centre Hospitalier de Villefranche

Villefranche-sur-Saône, 69400, France

RECRUITING

Médipôle de Villeurbanne

Villeurbanne, 69100, France

RECRUITING

MeSH Terms

Conditions

Arthritis, Juvenile

Condition Hierarchy (Ancestors)

Arthritis; Joint Diseases; Musculoskeletal Diseases; Rheumatic Diseases; Connective Tissue Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases

Central Study Contacts

Yvan Jamilloux, Dr

CONTACT

+33426732636; yvan.jamilloux@chu-lyon.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 8, 2021
• First Posted: September 24, 2021
• Study Start: February 1, 2020
• Primary Completion: February 1, 2025
• Study Completion: December 1, 2025
• Last Updated: April 26, 2024

Record last verified: 2024-04

Data Sharing

• IPD Sharing: Will not share

Locations

FR (16)