Evidence map›Paper›PMID 40420428›Full record

ArticleArthritis & rheumatology (Hoboken, N.J.)2025

Impact of Interleukin-1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR/ACR/PRINTO 2016 MAS Classification Criteria.

Remco G A Erkens, Greta Rogani, Laura Huber, Anouk Verwoerd, Dieneke Schonenberg-Meinema, J Merlijn van den Berg, Wineke Armbrust, G Elizabeth Legger, Sylvia Kamphuis, Ellen J H Schatorjé and 5 more

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Article in Arthritis & rheumatology (Hoboken, N.J.), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing 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

2 citing papers in PubMed.

  1. Article
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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

15 authors.

Remco G A ErkensDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital and Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.ORCID https://orcid.org/0000-0002-3962-7228
Greta RoganiDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital and Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Laura HuberDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Anouk VerwoerdDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital and Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Dieneke Schonenberg-MeinemaDivision of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.
J Merlijn van den BergDivision of Pediatric Immunology, Rheumatology and Infectious Diseases, Emma Children's Hospital, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.
Wineke ArmbrustDivision of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, University Medical Center Groningen, Groningen, The Netherlands.
G Elizabeth LeggerDivision of Pediatric Rheumatology and Immunology, Beatrix Children's Hospital, University Medical Center Groningen, Groningen, The Netherlands.ORCID https://orcid.org/0000-0002-9576-0904
Sylvia KamphuisDivision of Pediatric Rheumatology, Sophia Children's Hospital, Erasmus Medical Center, Rotterdam, The Netherlands.
Ellen J H SchatorjéDepartment of Pediatric Rheumatology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Esther P A H HoppenreijsDepartment of Pediatric Rheumatology, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Joost F SwartDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Marc H A JansenDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Jorg van LoosdregtCenter for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
Sebastiaan J VastertDivision of Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital and Center for Translational Immunology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo evaluate the applicability of the 2016 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR)/Paediatric Rheumatology International Trials Organisation (PRINTO) macrophage activation syndrome (MAS) classification criteria in patients with Still disease and systemic juvenile idiopathic arthritis (sJIA-SD) treated with interleukin-1 (IL-1)-targeted therapy and to assess the incidence of MAS in this context.

methodsWe analyzed retrospective and prospective data from Dutch patients with sJIA-SD (diagnosis 2008-2017, n = 54) and data from a nationwide prospective Dutch cohort and intervention study (diagnosis 2017-2022, n = 66). From these cohorts, MAS episodes developing in patients with sJIA-SD treated with IL-1-targeted therapy (anakinra or canakinumab) with at least two years of follow-up were selected. Clinical and laboratory data were extracted from the electronic patient files.

resultsA total of 22 patients experienced 29 MAS episodes while receiving IL-1-targeted treatment. Seven patients had recurrent MAS episodes (not all while receiving IL-1 blockade). The 2016 criteria for MAS in sJIA-SD were met for 28 of 29 MAS episodes (97%). Within the prospective nationwide cohort starting anakinra as first-line monotherapy, the incidence rate of MAS in the first two years of disease was 18% (12 of 66 patients, with 11 of 12 while receiving IL-1 inhibition). This incidence is comparable to that observed in historical glucocorticoid-treated patients. Half of MAS episodes occurred within three months after diagnosis, and Epstein-Barr virus was the most common identifiable trigger.

conclusionAlthough first-line anakinra in new-onset sJIA-SD has demonstrated high response rates, our data suggest the incidence of MAS in the first two years of disease is not reduced. Patients appear to be particularly at risk early in disease. Importantly, our data show that the EULAR/ACR/PRINTO 2016 MAS classification criteria remain applicable to patients receiving IL-1-targeted therapy.

Indexed as

Antirheumatic AgentsArthritis, JuvenileInterleukin-1Interleukin 1 Receptor Antagonist ProteinMacrophage Activation SyndromeAdolescentAdultAntibodies, Monoclonal, HumanizedChildFemaleHumansIncidenceMaleNetherlandsProspective StudiesRetrospective StudiesAntibodies, Monoclonal, HumanizedAntirheumatic AgentscanakinumabInterleukin-1Interleukin 1 Receptor Antagonist Protein

Identifiers

PMID40420428
PMCPMC12750127

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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.