Spring 2026 Press Review – Paediatric rheumatology

December 2025 to March 2026

Author: Sonia Melo Gomes

Multi-omics analysis identifies neutrophil extracellular traps-associated candidate genes in IgA vasculitis

Lin et al. performed multi-omics analysis on peripheral blood bulk transcriptomic data from patients with IgA vasculitis and healthy controls, focusing on the differential expression of a set of 646 NET-associated genes. Different analyses were intersected for candidate gene prioritization.
Five NET-associated candidate genes—AGER, TLR2, CXCR2, TEK, and THBD—were consistently prioritized across analyses. Mendelian randomization results indicated that higher expression of AGER, TLR2, and CXCR2 was associated with increased IgAV risk, whereas TEK and THBD showed inverse associations. These results need to be validated in larger studies and with functional studies.

Biomarkers for recalcitrating disease course in children with Kawasaki disease/ MIS-C

Satirer et al. analysed data from a cohort of 80 children with hyperinflammation in order to identify early biomarkers predictive of recalcitrant disease course (56-70%). Rash, mucosal involvement, and conjunctivitis were more common in the monophasic group, while abdominal pain, neurological signs, pleural effusion, lymphopenia, thrombocytopenia, hypoalbuminemia, and elevated ferritin characterised the recalcitrant group (p < 0.01). Myocarditis and reduced ejection fraction occurred only in the recalcitrant group; coronary artery changes were more frequent in monophasic cases (21% vs. 5%).
NT-proBNP, IL-2R, and SAA were strongly associated with a recalcitrant hyperinflammatory course and may aid early prognostication and monitoring.

CARRA survey identifies variability in vaccination practices in children with rheumatological diseases

De Souza et al. conducted a 44-question survey about vaccine practices amongst the CARRA network aiming to understand provider approach to vaccine review/ reconciliation and identify possible barriers to immunization.
Up to 90% of responders endorsed diverse approaches to vaccine review. Immunosuppressant changes prompted providers to assess baseline receipt of live vaccines over non-live vaccines (p < 0.001).
Provider-identified barriers to immunization included lack of clinic resources (58%), patient or parent vaccine hesitancy (27%), and family concerns regarding vaccines (20%).
Standardized vaccine review practices and improved communication with primary care providers can optimise opportunities to improve vaccination rates and increase awareness.

Use of iv anakinra in paediatric patients with inflammatory conditions seems to have a good safety profile

Klanjscek et al. conducted a multicenter, retrospective cohort study of 113 children treated with intravenous anakinra: 64 (56.6%) had underlying rheumatologic diseases, 27 (23.9%) had onco-hematologic diseases, 22 (19.5%) had severe systemic infections.
Adverse events were observed in 10 children (8.8%). The most common events were transient elevation of liver or pancreatic enzymes (6.2%) and maculopapular rash (1.2%). One patient (0.9%) experienced an anaphylactoid reaction. Sixteen patients (14.2%) died. The rheumatological disease subgroup comprised cases of HLH, MAS, Kawasaki disease, and MIS-C. The mean anakinra dose was 8.38 mg/kg/day (range 2.2-20 mg/kg/day). There were no deaths in this subgroup.

Delays over 6 months in the diagnosis of juvenile idiopathic arthritis impact service use, health-related costs and quality of life

Von Huben et al. performed a cost analysis study over a lifetime horizon comparing the time from actively seeking treatment to formal diagnosis (< 6 months, 6 + months) to quantify the impact of diagnostic delays on health service use, health-related quality of life (HRQoL) and associated costs. The group diagnosed within 6 months (n=102/163) had a lower use of health services, resulting in a mean annual decrease in costs >AUD$10,000 per child. Most of the cost savings were due to reductions in hospitalizations for pain, inflammation, and investigative procedures. There were also significant increases in HRQoL, which persisted over 20 years.

PRO-KIND group establishes consensus protocol for classification, monitoring and therapy in persistent oligoarticular juvenile idiopathic arthritis

Peitz et al. formulated a group of 23 statements concerning the management of persistent oligoarticular JIA. These statements were circulated as survey amongst medical members of the  German Society for Paediatric and Adolescent Rheumatology achieving a 65% response rate (80/124). Overall agreement with the statements was between 87.7-100%. Comments and proposals for change were discussed in the working group and incorporated into the statements where appropriate, resulting in a total of 20 statements being agreed upon.
The resulting statements focus on diagnosis (1-2), diagnostic and monitoring (3-9) and treatment (10-20).

Superb microvascular imaging (doppler) ultrasound of the knee in JIA patients shows excellent repeatability measures for assessment of synovial vascularity

Dohna et al. conducted a prospective multicenter repeatability study for assessment of intra and inter-observer reliability of the novel doppler technique Superb Microvascular Imaging (SMI). The 76 children with JIA included in the study underwent 3 standardized SMI scans, 2 by the same examiner and a third by a different one. Intra-observer reliability was excellent (ICC = 0.972, 95% CI: 0.956–0.982). Inter-observer reliability was strong (ICC = 0.828–0.928), regardless of examiner experience.
Agreement between imaging planes was substantial (κ = 0.72, p = 0.32). Synovial vascularity scores correlated significantly with clinical measures of active arthritis (OR = 1.182, p = 0.0004), particularly with swelling (OR = 1.249, p < 0.0001).

A systematic literature review and metanalysis identifies risk factors for macrophage activation syndrome (MAS) in patients with systemic JIA (sJIA)

Li et al. conducted a systematic literature review on publication databases and subsequent meta-analysis of case-control studies investigating risk factors for MAS in sJIA. A total of 10 studies involving 1,936 patients were included, comprising 643 cases and 1,293 controls. Meta-analysis showed that central nervous system (CNS) involvement, hypofibrinogenemia, markedly elevated ferritin levels, and leukopenia were significantly associated with MAS in sJIA, with odds ratios (95% confidence intervals) of 4.30 (2.13–8.65), 4.03 (2.87–5.65), 8.28 (5.66–12.10), and 5.98 (2.80–12.75), respectively.

Analysis of multicenter vs single-center cohorts shows discordance between rates of non-zero physician’s global scores with absence of active joints in JIA

Ribollo-Gimenez et al. performed a study comparing the frequency of instances in which the physician’s global assessment of disease activity (PhGA) was scored >0 despite the absence of active joints in children with JIA, using two multicenter patient datasets and one single-center dataset from a paediatric rheumatology center with expertise in clinimetric assessments. The percentage of PhGA scores >0 as the sole unmet clinical Inactive disease (CID) criterion was 14.8% /13.7% in the multicenter datasets and 5.1% in the single-center dataset. The CID criteria most frequently unmet for PhGA scores>0 were elevated acute-phase reactants and morning stiffness lasting ≥15min.