Clinical Autoinflammatory Disease Cohort
Trial statusNot yet recruiting
Trial phaseNot listed
Trial typeObservational
Biological sexAll
Age18-75
SponsorRenJi Hospital
About this trial
Autoinflammatory diseases (AIDs) are a group of rare disorders caused by dysregulation of the innate immune system, characterized by recurrent fever, systemic inflammation, and involvement of specific organs. Diagnosis relies on a combination of clinical features, laboratory tests, genetic findings, and response to treatment. Still's disease is a representative type of AID, marked by high spiking fevers, polyarthritis, evanescent rash, and markedly elevated inflammatory markers. Among its complications, macrophage activation syndrome (MAS) is the most life-threatening, affecting approximately 10-15% of patients. MAS involves uncontrolled immune activation and systemic inflammation, and if left untreated, may result in a mortality rate exceeding 50%. This study aims to develop a standardized clinical dataset and diagnostic-treatment framework for AIDs based on their disease characteristics. After establishing a data collection template, eligible patients aged 18-75 years with AIDs will be enrolled. Clinical data will be collected to build a prospective follow-up cohort, focusing particularly on adult-onset Still's disease (AOSD), to explore the clinical features and pathogenesis of AIDs.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Diagnosis of adult-onset Still's disease (AOSD) according to the Yamaguchi criteria (as described above) or other established autoinflammatory disease diagnostic criteria.
Age between 18 and 75 years, inclusive.
Willingness and ability to comply with scheduled follow-up visits, examinations, and treatments.
Voluntary provision of written informed consent.
Disqualifiers
Refusal or inability to provide informed consent; age under 18 or over 75 years; inability to provide at least 80% of the required core data for Still's disease or other autoinflammatory diseases.
Presence of other connective tissue diseases.
Positive detection of autoantibodies.
Current acute infection or history of active tuberculosis.
Trial population
Adult patients (aged 18-75 years) diagnosed with autoinflammatory diseases.
Trial design
Design model
Cohort
Time perspective
Other
Treatments tested in this trial
Not applicable- observational study
Other interventionno interventions were involved
Treatment groups
1,000 Participants
are divided into 1 treatment groupGroup A: AIDs patients1 intervention
Trial outcomes
Primary outcomes
1
Sustained Event-Free Remission
Proportion of participants who maintain clinical remission without experiencing disease-related complications or treatment-related adverse events, including glucocorticoid-associated toxicity.
Time frame
From the date of remission until the first documented disease progression, occurrence of treatment-related severe adverse events, or death from any cause, whichever occurred first, assessed up to 60 months.Secondary outcomes
1
Glucocorticoid discontinuation rate
Proportion of patients who successfully taper and discontinue glucocorticoids
Time frame
3 months2
Time to biologic DMARD initiation
From enrollment to the start of biologic DMARD therapy
Time frame
From enrollment to 36 months3
Time to first MAS event
Time from enrollment to first documented macrophage activation syndrome
Time frame
From enrollment to 3 months4
Time to Onset of Glucocorticoid-Associated Complications
Time interval between the start of glucocorticoid treatment and the occurrence of the first treatment-related adverse event, including but not limited to infections, osteoporosis, hypertension, or diabetes.
Time frame
From initiation of glucocorticoid therapy to the first documented complication, with follow-up of up to 60 months.Locations
Sponsors and contacts
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Source ClinicalTrials.gov
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