Evidence map›Paper›PMID 41281302›Full record

ReviewRheumatology advances in practice2025

Pharmacological and non-pharmacological treatments for refractory paediatric Still's disease: a scoping review.

Elizabeth Twynam-Perkins, Neil Martin, Kirsty McLellan

Abstract readReview
In one paragraph

Review in Rheumatology advances in practice, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors.

Elizabeth Twynam-PerkinsDepartment of Paediatric Rheumatology, Royal Hospital for Children, Glasgow, UK.ORCID https://orcid.org/0009-0003-7302-6893
Neil MartinDepartment of Paediatric Rheumatology, Royal Hospital for Children, Glasgow, UK.
Kirsty McLellanDepartment of Paediatric Rheumatology, Royal Hospital for Children, Glasgow, UK.ORCID https://orcid.org/0000-0003-3835-427X

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Objectives: Management of systemic juvenile idiopathic arthritis, or Still's disease, has been transformed by the introduction of IL-1 and IL-6 antagonists. Despite this, a minority have refractory disease with three subtypes described: (1) persistent arthritis, (2) recurrent or difficult-to-treat macrophage activation syndrome (MAS), or (3) interstitial lung disease. This scoping review aimed to summarize available evidence for the treatment of refractory paediatric Still's. Methods: Databases were searched using terms 'systemic JIA' or 'Still's disease' AND 'refractory', plus synonyms. Records were screened for papers on the treatment of refractory Still's, before reviewing full texts. Controlled trials, uncontrolled trials and case series/reports involving patients with disease onset <16 years were included. Data were extracted in tabulated form for study characteristics and outcome measures (survival, disease remission, reduction in corticosteroids, and adverse effects). Quality assessment was performed using the appropriate JBI checklist. Results: Thirty articles matched the inclusion criteria: 1 pilot study, 8 case series and 21 case reports. No controlled studies were identified with all three subtypes of refractory Still's represented. There were positive results in a pilot study of emapalumab (IFN-gamma inhibitor) for the treatment of refractory MAS, and beneficial results with combination biologics and Janus kinase (JAK) inhibitors for refractory Still's arthritis. There are reports of successful management with allogenic stem cell transplantation, although with significant risks. Conclusions: Various treatment strategies are reported in refractory Still's, including emapalumab, JAK inhibitors, rituximab and combination biologics, although the evidence base is currently limited. Consistent outcome reporting and innovative trial designs are required to provide further evidence in these challenging subgroups.

Indexed as

interstitial lung diseasemacrophage activation syndromerefractoryStill’s diseasesystemic juvenile idiopathic arthritis

Identifiers

PMID41281302
PMCPMC12631771

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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.