An observational study in Still's Disease, Adult-Onset, Systemic Inflammation and Arthritis, sponsored by National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). Recruiting at 1 site in United States. Open to participants aged 1 Day to 100 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2026-10-22.
Sponsored by National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) · Observational
Background:
Inflammatory conditions can cause symptoms like fevers, arthritis, and rash. Systemic juvenile idiopathic arthritis (sJIA) is one of these conditions. So is adult-onset Still s disease (AOSD). Their causes are unknown. Researchers want to learn more about these conditions. This includes genetic changes and environmental factors.
Objective:
To study sJIA and AOSD in children and adults over time.
Eligibility:
People with known or suspected sJIA, AOSD, or similar inflammatory condition
Design:
Participants will be screened with a phone call.
Participants will have 1 visit. It may be outpatient or they may be admitted to the clinic. The visit may last up to 5 days. Participants will have:
Participants may also have:
Participants who have a joint aspiration may provide a fluid sample. The joint will be prepared, then fluid is removed by needle. A corticosteroid may be injected.
Participants who have a bone marrow biopsy may provide sample cells.
Participants may be seen by NIH specialists.
Members of the participant s family and healthy volunteers may give blood or saliva samples for genetic testing.
Participants may repeat some study tests every 6 months.
Study Description:
The purpose of this protocol is to study the natural history, genetics and pathophysiology of systemic juvenile idiopathic arthritis (sJIA), adult-onset Still s disease (AOSD) and related inflammatory conditions. One of seven subtypes of juvenile idiopathic arthritis (JIA), sJIA contributes disproportionately to the morbidity and mortality of JIA and is considered by many to be the most severe JIA subtype. sJIA typically presents with fever of unknown origin and arthritis, together with evanescent skin rash, serositis, hepatosplenomegaly and lymphadenopathy. It is strongly associated with macrophage activation syndrome (MAS), which has a high fatality rate when untreated. AOSD is phenotypically similar to sJIA in presentation, progression and association with MAS, however it develops after the 16th birthday. The causes sJIA and AOSD are poorly understood.
sJIA and AOSD are diagnoses of exclusion and there are often delays in their diagnosis due to the stringency of their classification criteria. There is no diagnostic test for sJIA/AOSD and there exists significant overlap with other conditions. The manifestations and severity of disease can differ among patients, further delaying the diagnosis. There is also considerable variability in both patient response to therapy and long-term outcomes, and there exist no therapeutic or prognostic biomarkers to guide treatment.
Given our limited understanding of the causes, treatments and prognostic factors of sJIA, we developed this protocol to longitudinally follow patients with sJIA/AOSD and investigate these topics. The specific goals of this protocol include: 1) Establishing a cohort of patients with sJIA/AOSD and assembling a detailed set of longitudinal clinical information; 2) Identifying genetic factors that cause or influence susceptibility to sJIA/AOSD; 3) Determining the functional relevance of genes and variants that influence sJIA/AOSD; and 4) Developing a molecular library of patient biological samples which may be used to further investigate sJIA/AOSD.
Patients enrolled in this protocol will undergo screening history, physical examination and laboratory evaluation. At times, we may ask for permission to evaluate additional family members. We will collect peripheral blood samples for genetic and functional studies from affected patients, unrelated healthy volunteers and in some cases patients' family members. We will ask permission to perform whole genome/exome sequencing. We also may ask some patients to undergo skin biopsy for research purposes. This study aims to elucidate genetic factors that contribute to sJIA/AOSD and related conditions and to determine their implications on inflammatory pathophysiology. By so doing, we hope to identify novel therapeutic targets for inflammatory disease.
Objectives:
The intent of this natural history protocol is to recruit a cohort of patients with sJIA, AOSD and related inflammatory conditions to prospectively follow, who over the course of several years will help us to better understand the potential triggers for disease development, relapse, and worsening. Over time, we will test many hypotheses. Specifically, we will aim to identify genetic polymorphisms in the sJIA and AOSD population which may influence disease susceptibility. We also hope to identify differential expression and function of these susceptibility genes and in so doing, explain disease pathogenesis. The information collected may also be helpful in identifying biomarkers to help diagnose disease. With this protocol, we also aim to identify possible therapeutic targets for treatment of disease. Given the large amount of clinical data generated, it may be possible to identify distinct subclasses of sJIA and AOSD which can be used to further classify patients and thereby better predict prognosis and tailor the most appropriate treatment plan. This cohort will be invaluable for further research as well by providing a molecular library of biological material and cell lines which can be used for future research projects.
Specific objectives are as follows:
To identify patients with sJIA, AOSD, and similar phenotypes for possible entry into future treatment protocols
23 studies on the registry are indexed under Still's Disease, Adult-Onset; 5 are open to participants now.
This study's planned enrollment of 2,000 is above the median of 155 across 10 observational studies indexed under Still's Disease, Adult-Onset.
Browse Still's Disease, Adult-Onset studies →National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) is the lead sponsor of 100 studies on the registry; 12 are open to participants now.
Of its 7 completed or terminated interventional studies of FDA-regulated products, 5 (71%) have results posted.
Counted across the registry records on this site, refreshed daily.
The criteria were revised on the registry on Sep 25, 2026. See what changed
Patients with systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD) and related inflammatory conditions.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Affected subject:
Patients with signs and symptoms of sJIA will be classified as outlined in Diagnosis/Classification Criteria.
Patients with signs and symptoms of AOSD will be classified as outlined in Yamaguchi Criteria for diagnosing Adult-Onset Still's Disease.
Healthy Subjects:
Healthy Volunteer should not have any history of , autoinflammatory, rheumatic or proliferative/malignant disease or any unexplained inflammatory signs/symptoms or conditions (applies to unrelated).
EXCLUSION CRITERIA:
Affected and Healthy subjects:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
A biopsy takes a small piece of tissue to examine. “Archival tissue” is a sample already taken, for example at diagnosis, and kept by the hospital.
Whether a stored sample will do depends on when it was taken, how much there is and what the study needs. Ask whether the study accepts your stored sample or needs a new biopsy.
A condition in which the immune system attacks the body’s own tissues, such as rheumatoid arthritis, lupus, psoriasis, type 1 diabetes or inflammatory bowel disease.
Immunotherapy studies often leave out active autoimmune disease, and some name exceptions, such as thyroid disease on replacement hormone. Check the exceptions this study lists.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Biopsy and archival tissue
A biopsy takes a small piece of tissue to examine. “Archival tissue” is a sample already taken, for example at diagnosis, and kept by the hospital.
Whether a stored sample will do depends on when it was taken, how much there is and what the study needs. Ask whether the study accepts your stored sample or needs a new biopsy.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Autoimmune disease
A condition in which the immune system attacks the body’s own tissues, such as rheumatoid arthritis, lupus, psoriasis, type 1 diabetes or inflammatory bowel disease.
Immunotherapy studies often leave out active autoimmune disease, and some name exceptions, such as thyroid disease on replacement hormone. Check the exceptions this study lists.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Composed of patients with known or suspected AOSD as defined by Yamaguchi criteria.
Composed of family members of patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease and related conditions.
Composed of healthy adults and children (above the age of 6 years) who volunteer to participate in this protocol.
Composed of patients with suspected inflammatory disease as indicated by the presence of episodic fever and/ or arthritis.
Composed of patients with known or suspected sJIA as defined by the international league of Associations for Rheumatology (ILAR) criteria
Genetic sequencing of patients
sequencing aims to identify variants to help stratify sJIA, Still's and inflammatory disease patients
Time frame: Ongoing
From the registry record's own update history. This site started tracking changes on Sep 25, 2026; for anything earlier, see the record history on ClinicalTrials.gov ↗
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Still's Disease, Adult-Onset →
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