Still DiseaseCAIDNCT07126145
Clinical Autoinflammatory Disease Cohort
An observational study of Still Disease and Autoinflammatory Disease, run by RenJi Hospital.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 18 to 75 years
- Conditions the study is for
- Still Disease, Autoinflammatory Disease
- What is being tested?
- Not applicable- observational study
- How many people
- 1,000 participants
- Where
- No sites listed yet
- Study dates
- Aug 2025to Aug 2035
- How long will I take part?
- 12 Months
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 18 to 75 years
- All sexes
- No healthy volunteers
- Adult, Older adult
Study population: Adult patients (aged 18-75 years) diagnosed with autoinflammatory diseases.
Who may be able to join
- 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.
Who cannot join
- 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.
- History of allergic constitution or multiple drug allergies.
- Psychiatric disorders or other conditions preventing compliance with examinations, follow-up, or treatment.
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- Women who are currently pregnant or planning to become pregnant.
- Presence of malignant tumors.
- Participation in other clinical trials currently or within a specified washout period.
About this study
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.
Read the detailed description
Autoinflammatory diseases (AIDs) are a group of rare disorders caused by overactivation of the body's innate immune system. They are marked by repeated episodes of fever, widespread inflammation, and damage to various organs. Because their symptoms can affect many different systems and often resemble other conditions, AIDs are frequently misdiagnosed or remain undetected for long periods.
The term "AIDs" covers more than 50 known single-gene (monogenic) diseases, as well as multi-gene (polygenic) conditions without an identified genetic cause. Examples include Still's disease, Behçet's disease, and certain types of inflammatory bowel disease. Adult-onset systemic autoinflammatory diseases (SAIDs) include Still's disease, Schnitzler syndrome, and idiopathic recurrent autoimmune pericarditis (IRAP). Common symptoms are periodic fevers of unknown origin, skin rashes, inflammation of membranes around organs (serositis), swollen lymph nodes, and joint pain or swelling. Laboratory findings often reveal elevated inflammatory markers such as ESR and CRP. These conditions can be further classified by their underlying pathways, including inflammasome-related diseases, type I interferonopathies, proteasome-related disorders, and NF-κB pathway-associated diseases.
Still's disease is a particularly rare type of SAID. It presents with high fevers, multiple joint pain, rash, and markedly increased inflammatory markers. The disease affects an estimated 2-6 people per million each year worldwide, but rates vary across populations. A serious complication, macrophage activation syndrome (MAS), develops in about 10-15% of patients. MAS involves uncontrolled immune activation and a massive release of inflammatory proteins known as cytokines-a process often called a "cytokine storm." Without rapid treatment, MAS can lead to multi-organ failure and has a mortality rate above 50%.
The exact cause of Still's disease and MAS remains unclear, but research shows it involves several intertwined processes: excessive immune cell activation, abnormal cytokine release, amplification of inflammation, and injury to multiple organs. Key inflammatory mediators include IL-1β, IL-6, IL-18, IFN-γ, and TNF-α. These molecules activate immune cells such as macrophages, neutrophils, and certain T cells, setting off a self-perpetuating cycle of inflammation. In addition, the formation of neutrophil extracellular traps (NETs) appears to play a role in tissue injury.
Because current diagnostic tools cannot reliably detect MAS early, and treatments-mainly glucocorticoids and immunosuppressants-are only partly effective and carry significant side effects, there is a pressing need for better understanding of the disease mechanisms. This study aims to follow a well-characterized cohort of patients with autoinflammatory diseases, particularly those with Still's disease and MAS, to identify new molecular targets and specific diagnostic biomarkers. The goal is to improve early detection, guide more precise treatments, and ultimately reduce mortality in these high-risk patients.
- Conditions
- Still Disease, Autoinflammatory Disease
- Being tested
- Not applicable- observational study
- Medical subject headings
- Arthritis, Juvenile
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Not applicable- observational studyOther
no interventions were involved
| Study group | Role | What they receive |
|---|---|---|
| AIDs patients | Patients with Still's disease must meet the 1992 Yamaguchi diagnostic criteria; patients with monogenic autoinflammatory diseases must meet the confirmed diagnostic criteria for the respective conditions; all patients must provide written informed consent.；Age range: 18-75 years old patients with autoinflammatory diseases. Receives: Other: Not applicable- observational study |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 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 (6)
Glucocorticoid discontinuation rate
Proportion of patients who successfully taper and discontinue glucocorticoids
Measured: 3 months
Time to biologic DMARD initiation
From enrollment to the start of biologic DMARD therapy
Measured: From enrollment to 36 months
Time to first MAS event
Time from enrollment to first documented macrophage activation syndrome
Measured: From enrollment to 3 months
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.
Measured: From initiation of glucocorticoid therapy to the first documented complication, with follow-up of up to 60 months.
Incidence of Treatment-Related Adverse Events
Number and proportion of participants who experience one or more adverse events attributable to treatment during the follow-up period. Adverse events may include infections, hepatic dysfunction, cytopenia, or other complications associated with immunosuppressants, glucocorticoids, or biologic agents.
Measured: From treatment initiation to 36 months
Time to Relapse After Remission
Duration between the time a participant first achieves clinical remission and the time of first subsequent disease relapse, defined by pre-specified clinical or laboratory criteria.
Measured: From achievement of remission to first documented disease flare, with follow-up of up to 60 months.
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted17 Aug 2025
- Study startAug 2025 (estimated)
- Primary completionAug 2030 (estimated)
- Study completionAug 2035 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Still DiseaseStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Other
- Enrolment
- 1,000 participants (estimated)
- Target duration
- 12 Months
Sponsor and investigators
- Lead sponsor
- RenJi Hospital
- Sponsor type
- University, hospital or other
- Responsible party
- Principal investigator, Qiong Fu, RenJi Hospital
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07126145
- Sponsor's protocol ID
- LY2025-170-A
- Acronym
- CAID
- Status last verified
- Aug 2025
- First submitted
- 2 Aug 2025
- Last updated
- 17 Aug 2025
- Results posted
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
Study at a glance
From the registry record, last updated by the sponsor on 17 Aug 2025. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Clinical Autoinflammatory Disease Cohort
- ClinicalTrials.gov ID
- NCT07126145
- Acronym / sponsor ID
- CAID / LY2025-170-A
- Conditions
- Still Disease, Autoinflammatory Disease
- Study type
- Observational
- Recruitment status
- Not yet recruiting
- Enrolment
- 1,000 participants (estimated)
- Lead sponsor
- RenJi HospitalUniversity, hospital or other
- Study start
- Aug 2025 (estimated)
- Primary completion
- Aug 2030 (estimated)
- Study completion
- Aug 2035 (estimated)
- First posted
- 17 Aug 2025 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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.
Detailed description
Autoinflammatory diseases (AIDs) are a group of rare disorders caused by overactivation of the body's innate immune system. They are marked by repeated episodes of fever, widespread inflammation, and damage to various organs. Because their symptoms can affect many different systems and often resemble other conditions, AIDs are frequently misdiagnosed or remain undetected for long periods.
The term "AIDs" covers more than 50 known single-gene (monogenic) diseases, as well as multi-gene (polygenic) conditions without an identified genetic cause. Examples include Still's disease, Behçet's disease, and certain types of inflammatory bowel disease. Adult-onset systemic autoinflammatory diseases (SAIDs) include Still's disease, Schnitzler syndrome, and idiopathic recurrent autoimmune pericarditis (IRAP). Common symptoms are periodic fevers of unknown origin, skin rashes, inflammation of membranes around organs (serositis), swollen lymph nodes, and joint pain or swelling. Laboratory findings often reveal elevated inflammatory markers such as ESR and CRP. These conditions can be further classified by their underlying pathways, including inflammasome-related diseases, type I interferonopathies, proteasome-related disorders, and NF-κB pathway-associated diseases.
Still's disease is a particularly rare type of SAID. It presents with high fevers, multiple joint pain, rash, and markedly increased inflammatory markers. The disease affects an estimated 2-6 people per million each year worldwide, but rates vary across populations. A serious complication, macrophage activation syndrome (MAS), develops in about 10-15% of patients. MAS involves uncontrolled immune activation and a massive release of inflammatory proteins known as cytokines-a process often called a "cytokine storm." Without rapid treatment, MAS can lead to multi-organ failure and has a mortality rate above 50%.
The exact cause of Still's disease and MAS remains unclear, but research shows it involves several intertwined processes: excessive immune cell activation, abnormal cytokine release, amplification of inflammation, and injury to multiple organs. Key inflammatory mediators include IL-1β, IL-6, IL-18, IFN-γ, and TNF-α. These molecules activate immune cells such as macrophages, neutrophils, and certain T cells, setting off a self-perpetuating cycle of inflammation. In addition, the formation of neutrophil extracellular traps (NETs) appears to play a role in tissue injury.
Because current diagnostic tools cannot reliably detect MAS early, and treatments-mainly glucocorticoids and immunosuppressants-are only partly effective and carry significant side effects, there is a pressing need for better understanding of the disease mechanisms. This study aims to follow a well-characterized cohort of patients with autoinflammatory diseases, particularly those with Still's disease and MAS, to identify new molecular targets and specific diagnostic biomarkers. The goal is to improve early detection, guide more precise treatments, and ultimately reduce mortality in these high-risk patients.
Study design
- Observational model
- Cohort
- Time perspective
- Other
- Target follow-up
- 12 Months
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| AIDs patients | – | Other: Not applicable- observational studyPatients with Still's disease must meet the 1992 Yamaguchi diagnostic criteria; patients with monogenic autoinflammatory diseases must meet the confirmed diagnostic criteria for the respective conditions; all patients must provide written informed consent.；Age range: 18-75 years old patients with autoinflammatory diseases. |
Who may qualify
- Ages
- 18 to 75 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Adult, Older adult
- Study population
- Adult patients (aged 18-75 years) diagnosed with autoinflammatory diseases.
Key inclusion criteria (4 listed)
- 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.
Key exclusion criteria (9 listed)
- 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.
- History of allergic constitution or multiple drug allergies.
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Not applicable- observational studyno interventions were involved | Other | AIDs patients |
Primary outcome
Sustained Event-Free Remission
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.
Proportion of participants who maintain clinical remission without experiencing disease-related complications or treatment-related adverse events, including glucocorticoid-associated toxicity.
6 secondary outcomes
Glucocorticoid discontinuation rate
Time frame: 3 months
Time to biologic DMARD initiation
Time frame: From enrollment to 36 months
Time to first MAS event
Time frame: From enrollment to 3 months
Time to Onset of Glucocorticoid-Associated Complications
Time frame: From initiation of glucocorticoid therapy to the first documented complication, with follow-up of up to 60 months.
Incidence of Treatment-Related Adverse Events
Time frame: From treatment initiation to 36 months
Time to Relapse After Remission
Time frame: From achievement of remission to first documented disease flare, with follow-up of up to 60 months.
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 17 Aug 2025.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
The record lists no contact. Use the official record to reach the sponsor.
Questions about this trial
What is this clinical trial studying?
An observational study of Still Disease and Autoinflammatory Disease, run by RenJi Hospital.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 4 inclusion and 9 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: opening soon, as of the sponsor's last update on 17 Aug 2025. Status can change between updates, so confirm with the study contact before making plans.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Sustained Event-Free Remission (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.). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 17 Aug 2025; AzTrials last refreshed its copy on 30 Oct 2026.