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Still's Disease, Adult-Onset clinical trials

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NCT ID: NCT06405152 Recruiting - Clinical trials for Macrophage Activation Syndrome

Assessment of Macrophage Activation syndromE in STill's Disease

AMETHYST
Start date: September 27, 2023
Phase:
Study type: Observational

Assessment of Macrophage activation syndrome in STill's disease: retrospective chart analysis of patient History, Symptom resolution and Treatment characteristics

NCT ID: NCT05927454 Active, not recruiting - Clinical trials for Still's Disease, Adult-Onset

Acostill ( RaDiCo Cohort) (RaDiCo Acostill)

Acostill
Start date: July 11, 2017
Phase:
Study type: Observational

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 0.1/100,000 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 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. The various clinical questions posed by this disease are as follows: - Why does it differentially affect two age groups of the population? - Why is the clinical expression heterogeneous with pure systemic or articular forms, the frequency of SAM, and rare organ damage? - Why is the evolution over time different with resolving monocyclic forms or polycyclic forms and sometimes chronic evolutions? These 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. The 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. The expected outcomes of this work are to improve knowledge of Still disease and patient management on the following aspects: - Comparison of pediatric and adult forms (which has never been done on a large number of patients), - Better understanding of the pathogenic mechanisms of the disease, - The identification of early diagnostic/prognostic markers, - 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. The 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.

NCT ID: NCT05814159 Recruiting - Clinical trials for Still's Disease, Adult-Onset

A Study of Anakinra in Japanese Patients With Still's Disease (SJIA and AOSD)

Start date: August 24, 2022
Phase: Phase 3
Study type: Interventional

A study to demonstrate efficacy and safety of anakinra in pediatric and adult Japanese patients with Still's disease (Systemic juvenile idiopathic arthritis [SJIA] and Adult-onset Still's disease [AOSD]).

NCT ID: NCT05715736 Completed - Clinical trials for Still's Disease, Adult-Onset

Assessment of Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of APB-R3

Start date: March 8, 2023
Phase: Phase 1
Study type: Interventional

This will be a single centre, Phase 1, First-In-Human, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of Single Ascending Dose of APB-R3 in Healthy Participants.

NCT ID: NCT05432960 Withdrawn - AOSD Clinical Trials

Efficacy and Safety Clinical Study of RPH-104 in Adult Onset Still's Disease (AOSD)

Start date: December 2023
Phase: Phase 3
Study type: Interventional

The primary objective of the study is to evaluate the efficacy of RPH-104 when administered at a dose of 160 mg on Day 0, Day 7, Day 21 and then once every 2 weeks (Q2W) subcutaneous (SC) in patients with Adult Onset Still's Disease (AOSD). Furthermore, the study is scheduled to investigate pharmacokinetic (PK) and pharmacodynamic (PD) parameters of RPH-104.

NCT ID: NCT04752371 Terminated - Clinical trials for Adult Onset Still's Disease

A Study to Evaluate Camoteskimab in Participants With Still's Disease

Start date: March 25, 2021
Phase: Phase 1
Study type: Interventional

The main purpose of the study is to evaluate the safety and tolerability of Camoteskimab in participants with Still's Disease.

NCT ID: NCT04750863 Completed - Clinical trials for Adult Onset Still Disease

Evaluation of Adult Onset Still Disease Classification Criteria and Differential Diagnosis at Eighteen Months of Follow-up

AOSD-DIF
Start date: July 15, 2010
Phase:
Study type: Observational

Adult Onset Still Disease (AOSD) is an acquired inflammatory disease of unknown etiology, presenting with non specific symptoms. Its diagnosis rely on sets of criteria, i.e. Fautrel Criteria and Yamaguchi Criteria. However, differential diagnosis might appear during follow-up of patients. The aim of this study is to assess diagnostic performance of Fautral and Yamaguchi Criteria after 18 months of follow-up, and to describe differential diagnosis appearing during follow-up.

NCT ID: NCT04717635 Active, not recruiting - Clinical trials for Adult Onset Still's Disease

Study of Efficacy and Safety of Canakinumab in Japanese Patients With AOSD

Start date: March 30, 2021
Phase: Phase 3
Study type: Interventional

This is a phase III study designed to provide efficacy and safety data for canakinumab administered for at least 48 weeks as subcutaneous (s.c.) injection every 4 weeks (q4wk) in Japanese patients with Adult Onset Still's Disease (AOSD). Interim analysis (IA) data at Week 28 and 48 from this study supports a registration submission of canakinumab in the indication of Adult still's disease (ASD) in Japan.

NCT ID: NCT04402086 Recruiting - Clinical trials for Rheumatoid Arthritis

Rheumatology Patient Registry and Biorepository

Start date: August 4, 2020
Phase:
Study type: Observational [Patient Registry]

To facilitate clinical, basic science, and translational research projects involving the study of rheumatic diseases.

NCT ID: NCT03651193 Recruiting - Clinical trials for Adult Onset Still Disease

Multi-center Registration Study of Adult Onset Still's Disease in Chinese Population

MAOSDC
Start date: November 28, 2018
Phase:
Study type: Observational

This multi-center registration study will investigate the clinical characteristics of AOSD population in China , identify possible factors inducing the onset and active condition of AOSD in Chinese population , and identify the new high specific and sensitive markers of AOSD