Evidence map›Paper›PMID 42532550›Full record

SynthesisRMD open2026

Anakinra for intravenous immunoglobulin-resistant Kawasaki disease: a systematic literature review.

Caterina Matucci-Cerinic, Alessia Alunno, Jan W Schoones, Marco Gattorno, Isabelle Koné-Paut, Perrine Dusser

Abstract readSystematic Review
In one paragraph

Synthesis in RMD open, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

6 authors.

Caterina Matucci-CerinicUOC Rheumatology and Autoinflammatory Diseases, IRCCS Istituto Giannina Gaslini, Genova, Italy caterinamatuccicerinic@gaslini.org.ORCID http://orcid.org/0000-0003-3710-0569
Alessia AlunnoInternal Medicine and Nephrology Division, Department of Life, Health and Environmental Sciences, University of L'Aquila, L'Aquila, Italy.ORCID http://orcid.org/0000-0003-1105-5640
Jan W SchoonesDirectorate of Research Policy (formerly Walaeus Library), Leiden University Medical Center, Leiden, The Netherlands.ORCID http://orcid.org/0000-0003-1120-4781
Marco GattornoUOC Rheumatology and Autoinflammatory Diseases, IRCCS Istituto Giannina Gaslini, Genova, Italy.ORCID http://orcid.org/0000-0003-0704-1916
Isabelle Koné-PautDepartment of Pediatric Rheumatology, National Reference Centre for Auto-Inflammatory Diseases and Amyloidosis of Inflammatory Origin (CEREMAIA), Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), University of Paris Saclay Le Kremlin Bicêtre, Paris, France.ORCID http://orcid.org/0000-0001-8939-5763
Perrine DusserDepartment of Pediatric Rheumatology, National Reference Centre for Auto-Inflammatory Diseases and Amyloidosis of Inflammatory Origin (CEREMAIA), Bicêtre Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), University of Paris Saclay Le Kremlin Bicêtre, Paris, France.ORCID http://orcid.org/0000-0001-8118-0848

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundApproximately 10%-20% of patients with Kawasaki disease (KD) are resistant to intravenous immunoglobulins (IVIG) and are at increased risk of developing coronary artery aneurysms (CAA). A potential therapeutic role of anakinra (ANK) in refractory KD has been suggested. The aim of this work is to systematically review and critically appraise the available clinical evidence on the use of ANK in the treatment of IVIG-resistant KD, focusing on treatment indications, timing, dosage, efficacy on fever, inflammation, CAA and safety.

methodsA systematic literature search was conducted across PubMed, Embase, Web of Science, Cochrane Library, Emcare, Academic Search Premier and Google Scholar from inception to 9 October 2025, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines. Eligible studies included all study designs reporting outcomes of patients with KD treated with ANK.

resultsThirty-two publications (31 identified through database search and one by cross-referencing) describing 81 patients were included. Most reports were single cases or small series, with two early-phase clinical trials. Treatment with ANK was associated with resolution of fever and systemic inflammation in 94.9% of patients. Complete CAA resolution was reported in 32% and stabilisation/dimensional reduction in 56.3%. No serious safety concerns were identified. However, these findings derive from heterogeneous and low-quality evidence and should be interpreted with caution.

conclusionsIn IVIG-resistant KD, ANK was effective in controlling systemic inflammation, although more data are needed to assess its efficacy on coronary outcomes. While these findings are encouraging in supporting consideration of ANK for refractory KD, larger randomised clinical studies are warranted to define optimal ANK timing of introduction and dosing, and to evaluate its long-term efficacy on cardiac outcomes. PROSPERO REGISTRATION NUMBER: CRD420252129435.

Indexed as

Drug ResistanceImmunoglobulins, IntravenousInterleukin 1 Receptor Antagonist ProteinMucocutaneous Lymph Node SyndromeCoronary AneurysmHumansTreatment OutcomeImmunoglobulins, IntravenousInterleukin 1 Receptor Antagonist ProteinBiological TherapySystemic vasculitisTreatmentTumor Necrosis Factor Inhibitors

Identifiers

PMID42532550
PMCPMC13423158

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.