Evidence map›Paper›PMID 41033820›Full record

SynthesisThe Journal of rheumatology2026

Clinical Features and Outcome Measures Across Still Disease (Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still Disease) Cohorts Worldwide: A Systematic Literature Review.

Erin Balay-Dustrude, Mariana Correia Marques, Simone Appenzeller, Claudia Bracaglia, Fatma Dedeoglu, Esraa Eloseily, Penelope Martinez Jimenez, Michael J Ombrello, Karen Onel, Marinka Twilt and 4 more

Erratum issuedAbstract readSystematic Review
PubMed Full text at PMC Publisher
In one paragraph

Synthesis in The Journal of rheumatology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

14 authors.

Erin Balay-DustrudeE. Balay-Dustrude, MD, MS, S. Shenoi MBBS, MS, RhMSUS, Department of Pediatrics, Division of Rheumatology, University of Washington, Seattle, and Department of Pediatrics, Division of Rheumatology, Seattle Children's Hospital and Research Center, Seattle, Washington, USA; Erin.balay@seattlechildrens.org.ORCID https://orcid.org/0000-0001-6235-1947
Mariana Correia MarquesM. Correia Marques, MD, M.J. Ombrello, MD, Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.ORCID https://orcid.org/0000-0002-1195-1595
Simone AppenzellerS. Appenzeller, MD, Department of Orthopedics, Rheumatology, and Traumatology, University of Campinas, Campinas, and Rheumatology Laboratory, University of Campinas, Campinas, Brazil.ORCID https://orcid.org/0000-0001-5075-4474
Claudia BracagliaC. Bracaglia, MD, Division of Rheumatology, Bambino Gesù Children's Hospital, IRCCS, ERN-RITA Centre, Rome, Italy.ORCID https://orcid.org/0000-0002-9834-9619
Fatma DedeogluF. Dedeoglu, MD, Division of Immunology, Rheumatology Program, Boston Children's Hospital, Boston, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.ORCID https://orcid.org/0000-0003-2441-2803
Esraa EloseilyE. Eloseily, MD, MSc, University of Texas Southwestern Medical Center, Dallas, Texas, Scottish Rite for Children, Dallas, Texas, USA, and Assiut University Faculty of Medicine, Assiut, Egypt.ORCID https://orcid.org/0000-0002-4770-7383
Penelope Martinez JimenezP. Martinez Jimenez, MD, K. Onel, MD, Pediatric Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, New York, USA.ORCID https://orcid.org/0009-0001-4557-6228
Michael J OmbrelloM. Correia Marques, MD, M.J. Ombrello, MD, Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA.ORCID https://orcid.org/0000-0003-3322-4089
Karen OnelP. Martinez Jimenez, MD, K. Onel, MD, Pediatric Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine, New York, New York, USA.ORCID https://orcid.org/0000-0002-1238-5361
Marinka TwiltM. Twilt, MD, MScE, PhD, Department of Pediatrics, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.ORCID https://orcid.org/0000-0003-1954-2822
Xurong ZhaoX. Zhao, Library Services, Acute Care Alberta, Alberta Children's Hospital, Calgary, Alberta, Canada.
Francesca MinoiaF. Minoia, MD, Pediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID https://orcid.org/0000-0002-5093-8422
Susan ShenoiORCID https://orcid.org/0000-0002-2495-594X
CARRA Systemic JIA Work Group and the PReS MAS/sJIA Working Party

Funding

Genetics and pathophysiology of systemic juvenile idiopathic arthritis and other complex autoinflammatory diseasesZIAAR041198 · NIAMS · NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES · PI OMBRELLO, MICHAEL · 2014 to 2025
$14.4M
Intramural NIH HHS ZIA AR041198
6 · The paper itself

Abstract

objectiveMultinational research is essential to improve recognition and management of systemic juvenile idiopathic arthritis (sJIA). Current cohorts vary in the clinical variables and outcome measures collected. Adult-onset Still disease (AOSD) and sJIA are widely considered to comprise a single disease spectrum; however, classification criteria and clinical tools differ between groups. This systematic literature review aimed to identify clinical features and outcome measures collected across sJIA and AOSD cohorts worldwide to guide the development of a minimal dataset for Still disease.

methodsA literature search was conducted from 2000 to 2024 using Ovid MEDLINE, Embase, and Wiley Cochrane Library (Trials). Included articles were in English and described sJIA or AOSD cohorts of ≥ 20 patients, reporting patient characteristics, clinical and laboratory features, and outcome measures.

resultsA total of 240 articles were included (95 sJIA, 134 AOSD, 11 mixed), from 37 countries, describing 23,136 patients. International League of Associations for Rheumatology classification was used in 77.9% of sJIA studies, whereas 98.5% of AOSD studies used Yamaguchi criteria. There was no clear consensus on the definition of macrophage activation syndrome. Race and ethnicity were only reported in 11.7% of articles. Cohorts evaluated aligned on the most commonly collected laboratory items for both AOSD and sJIA, with some agreement among clinical features, whereas disease outcome measures used to evaluate and follow disease trajectory were variable.

conclusionData reporting across sJIA and AOSD cohorts for clinical characteristics and outcome measures is widely heterogeneous. Consensus on the identification of a standardized minimal dataset for Still disease cohorts is needed to foster future collaboration and improve patient outcomes.

Indexed as

Arthritis, JuvenileStill's Disease, Adult-OnsetAdultHumansOutcome Assessment, Health Careadult-onset Still diseaseclassification criteriacore setoutcomesystemic juvenile idiopathic arthritis

Identifiers

PMID41033820
PMCPMC12559961

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.