Paediatric recurrent pericarditis: Appropriateness of the standard of care and response to IL1-blockade

Objective: To analyse, in a cohort of paediatric patients with recurrent pericarditis (RP) undergoing anti-IL-1 treatment: the agent and dosing used as first line treatment, the long-term efficacy of IL1-blockers, the percentage of patients achieving a drug-free remission, the presence of variables associated with drug-free remission. Study design: Data were collected from patients' charts. Annualized relapse rate (ARR) was used for evaluation of treatment efficacy, bivariate logistic regression analysis for variables associated with drug-free remisison. Results: 58 patients, treated between 2008 and 2018, were included in the study (mean follow-up 2.6 years). 14/56 patients non-responsive to first line drugs were under-dosed. 57 patients were treated with anakinra: the ARR before and during daily treatment was 3.05 and 0.28, respectively (p<0.0001); an increase to 0.83 was observed after the reduction/withdrawal of treatment (p<.0001). The switch from anakinra to canakinumab (5 patients) was associated to an increase of the ARR (0.49 vs 1.46), but without statistical significance (p=0.215). At last follow-up only 9/58 patients had withdrawn all treatments. With the limits of a retrospective study and the heterogeneity between the patients enrolled in the study, a shorter duration of treatment with anakinra was the only variable associated with drug-free remission. Conclusion: This study shows that most of the pediatric patients with RP needing IL-1 blockade received an inadequate treatment with first line agents. The effectiveness of anakinra is supported by this study, but few patients achieved drug free-remission. The different rate of response to anakinra and canakinumab may suggest a possible role of IL1α in the pathogenesis of RP

Reliability of LoSCAT score for activity and tissue damage assessment in a large cohort of patients with Juvenile Localized Scleroderma

Abstract Objectives To assess reliability of the two indexes of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Skin Damage Index (LoSDI), when applied by clinicians with different experience in scoring and managing patients with JLS. Secondary aim was to compare LoSCAT and infrared thermography (IRT) in monitoring lesions over time. Methods Consecutive children with Juvenile Localized Scleroderma (JLS) were blindly evaluated by three examiners with different experience in Paediatric Rheumatology and with no experience in LoSCAT use. At each visit, patients were assessed by LoSCAT and IRT. Sensitivity to change of LoSCAT and IRT was assessed in a group of patients 3–6 months later. Inter-rater reliability was assessed by Intraclass Correlation Coefficient (ICC) and variance analysis (ANOVA). Findings Forty-seven patients (129 lesions) entered the study, and 26 (79 lesions) were re-evaluated with same modality after 4.5 (SD 1.5) months. mLoSSI showed excellent inter-rater reliability expressed by ICC 0.965 confirmed by ANOVA. Similarly, inter-rater reliability for LoSDI was good (ICC = 0.774) but worse concordance among examiners was observed. A comparable improvement of mLoSSI in all anatomic sites was noted by all examiners in 79 lesions examined in two subsequent visits and was consistent with thermography. Conclusions Different clinical experience in JLS did not influence clinical judgement in mLoSSI which showed excellent concordance, whereas LoSDI is less precise in damage assessment and not completely reliable in monitoring skin changes. Infrared thermography confirms to be a helpful tool for detecting disease activity and reliable in monitoring lesions over time

Methotrexate in Linear Scleroderma: long-term efficacy in 50 children from a single Pediatric Rheumatology Centre

OBJECTIVE: to study disease course and long-term outcome of children with Linear Scleroderma (LiS) treated with methotrexate (MTX) since diagnosis.METHODS: Retrospective and cross-sectional study including consecutive children with LiS treated with MTX for >1 year and with at least 2 years follow-up. Disease course was analyzed by number of relapses and treatment changes. Relapse-free survival (RFS) was examined by Kaplan-Meier analysis comparing patients with LiS and other JLS subtypes. Disease activity and damage were assessed by Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography.RESULTS: Fifty patients with mean follow-up 7.8 years and MTX treatment duration 3.1 years were included. Sixteen percent did not respond to first course of MTX and 16% had at least one flare. Complete remission was observed in 18.2% patients with 2-5 years follow-up, 80.0% by 10 years and 87.5% by >10 years. No significant difference in RFS between patients with LiS and 17 with other JLS subtypes was observed. Tissue damage was mild in 42% patients, moderate in 32% and severe in 26%. No significant correlation between severity of tissue damage and LiS subtype, disease duration, relapses and remission emerged. Treatment duration was significantly related to disease relapses (p<0.05) and severity of tissue damage (p<0.005).CONCLUSION: Most patients with LiS treated with MTX achieve complete and long-lasting remission. Overall aesthetic and functional sequelae are moderate, probably because tissue damage establishes early and treatment likely stabilizes it. Early diagnosis and MTX treatment as well as long-term monitoring are crucial to improve outcome and promptly identify flares