Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is diagnosed clinically, and inflammatory findings during an attack may resemble those of acute infection. We examined whether serum caspase-1, ASC/PYCARD, and copeptin change within the same child between attack and remission, and whether attack-phase levels differ from those seen in microbiologically confirmed febrile illness. We enrolled 50 children with PFAPA who provided paired attack and remission samples. Thirty-five children with microbiologically confirmed febrile illness and 26 healthy children served as comparison groups. The prespecified primary outcome was the within-child change in caspase-1, estimated on the log scale with a participant-clustered generalized estimating equation. Paired changes in ASC/PYCARD and copeptin were key secondary outcomes. Secondary models compared PFAPA attacks with febrile controls after adjustment for age, sex, fever-to-sampling interval, maximum temperature, and C-reactive protein. Median caspase-1 rose from 1.93 [1.60–2.78] ng/mL in remission to 3.96 [2.89–5.60] ng/mL during attacks (geometric mean ratio [GMR], 2.10; 95
Timely access to pediatric rheumatology (PR) care is critical for improving outcomes in juvenile idiopathic arthritis (JIA), yet, referral delays remain frequent. We aimed to examine the trajectories leading to PR care across two national healthcare settings. We conducted a multicenter study to characterize referral pathways to PR care among children with incident non-systemic JIA from the JIRcohort in France and Switzerland. We examined the sequence, number, and type of healthcare providers (HCPs) consulted before the first PR visit and assessed predictors of prolonged delays. Multiple correspondence analysis was used to explore patterns in referral trajectories. Among 227 patients, the median time from symptom onset to the first PR visit was 2.5 months [1.5; 6.3] in France and 3.0 months [1.2; 7.6] in Switzerland (p = 0.99). Referral pathways differed between the two settings, particularly in the number and type of HCPs involved, with fewer HCPs and more frequent pediatrician involvement in Switzerland. Multiple correspondence analysis identified four distinct referral pathway profiles combining clinical and referral characteristics, with differences in HCP involvement, clinical presentation, and referral time. A diagnosis of enthesitis-related arthritis and a higher number of HCPs involved were significantly associated with a longer time before the first PR visit (HR: 0.45, 95
Inclusion of stakeholders in the research priority setting is important to ensure patient-relevant research is produced. The aim of the study was to 1) conduct a systematic review to identify research priority setting activities in Juvenile Idiopathic Arthritis (JIA), and 2) compare research priorities identified by stakeholders in the review with those presented at recent pediatric rheumatology conferences. We conducted a systematic review of articles published on MEDLINE, Embase, PSYCINFO, and CINAHL between January 2010-May 2025. We included articles that identified stakeholder research priorities specifically for JIA, or included stakeholders that were predominantly JIA consumers. Data on the prioritization process were extracted using an appraisal checklist. The top five research priorities were collated into common topic areas and their ranking was examined according to each stakeholder group including patients, parents/carers and healthcare providers. Abstracts from conference proceedings for the European Pediatric Rheumatology Congress (PReS) (2023–2025) and the American College of Rheumatology (2023–2026) were examined and categorized into research priority groups. Of 127 studies identified, five studies examining broad research priorities for JIA were included. Priority topic areas identified were research into treatment and healthcare (30
The clinical significance of antiphospholipid antibody (aPL) profiles and persistence patterns in children is not well defined. This study aimed to characterize patterns of aPL positivity in children, and describe thrombotic and non-thrombotic manifestations, stratified by aPL profiles. In this single-center retrospective cohort study, we identified patients aged ≤ 18 years with at least one confirmed positive aPL result between 2003 and 2024. Persistent aPL positivity was defined as repeat positivity ≥ 12 weeks apart; transient positivity was defined as an initial positive result followed by a negative result on repeat testing. Antiphospholipid antibody profiles were classified as high-risk (lupus anticoagulant test [LA] positive, and/or anticardiolipin antibody [aCL] or anti–β2-glycoprotein-I antibody [aβ2GPI] IgG/IgM ≥ 40 U) or low-risk (LA negative with aCL and/or aβ2GPI IgG/IgM 20–39 U). Clinical features were compared using chi-square or Fisher’s exact test, as appropriate. Among 462 patients with documented initial aPL positivity, 246 (53
Children with rheumatic diseases are at elevated risk for vaccine-preventable diseases, yet vaccination rates in this population remain suboptimal. The specific barriers driving decreased immunization uptake in this population remain unclear. A convenience sample recruited through online caregiver support groups completed an exploratory survey assessing caregiver-reported immunization status along with vaccine hesitancy and other potential vaccine barriers among children with rheumatic disease. Univariable and multivariable logistic regression analyses evaluated factors associated with immunization uptake. Among 163 respondents, 64
