A protocol for scoping reviews on the role of whole-body and dedicated body-part magnetic resonance imaging for assessment of adult and juvenile idiopathic inflammatory myopathies.

Currently, standardized magnetic resonance imaging (MRI) scoring systems and protocols for assessment of idiopathic inflammatory myopathies (IIMs) in children and adults are lacking. Therefore, we will perform a scoping review of the literature to collate and evaluate the existing semi-quantitative and quantitative MRI scoring systems and protocols for the assessment and monitoring of skeletal muscle involvement in patients with IIMs. The aim is to compile evidence-based information that will facilitate the future development of a universal standardized MRI scoring system for both research and clinical applications in IIM. A systematic search of electronic databases (PubMed, EMBASE, and Cochrane) will be undertaken to identify relevant articles published between January 2000 and October 2023. Data will be synthesized narratively. This scoping review seeks to comprehensively summarize and evaluate the evidence on the scanning protocols and scoring systems used in the assessment of diagnosis, disease activity, and damage using skeletal muscle MRI in IIMs. The results will allow the development of consensus recommendations for clinical practice and enable the standardization of research methods for the MRI assessment of skeletal muscle changes in patients with IIMs.

Autoimmune inflammatory myopathy biomarkers.

The autoimmune inflammatory myopathy disease spectrum, commonly known as myositis, is a group of systemic diseases that mainly affect the muscles, skin and lungs. Biomarker assessment helps in understanding disease mechanisms, allowing for the implementation of precise strategies in the classification, diagnosis, and management of these diseases. This review examines the pathogenic mechanisms and highlights current data on blood and tissue biomarkers of autoimmune inflammatory myopathies.

Epidemiology of thromboembolic events in children and adolescents with antiphospholipid syndrome: A systematic review with meta-analysis.

BACKGROUND AND AIM: This was a systematic review and meta-analysis of the prevalence of thromboembolic events in children and adolescents with antiphospholipid syndrome (APS). METHODS: We searched PubMed, EMBASE and Web of Science to select relevant articles published between 1 January 2000 and 27 February 2022. We used the random-effects meta-analysis to estimate pooled point prevalence rates of thromboembolic events in studies with a minimum sample size of 30. RESULTS: We included five studies reporting data of 336 children and adolescents with primary APS and secondary APS (SAPS). Pooled point prevalence rates of initial general thrombosis, arterial thrombosis, venous thrombosis and stroke in individuals with seropositive APS were 98.2% (95% confidence interval [CI] 87.5-100), 27.6% (95% CI 21.4-34.2), 51.1% (95% CI 38.2-63.9) and 13.4% 95% CI (6.3-22.7), respectively. Pooled point prevalence rates of initial arterial and venous thromboses in children and adolescents with SAPS were 45.7% (95% CI 21.1-71.6) and 29.2% (95% CI 14.8-46), respectively. CONCLUSION: Arterio-venous thromboembolism is highly frequent in children and adolescents with SAPS. More studies using thrombotic and non-thrombotic APS classification criteria are warranted to better assess the frequency and predictors of thromboembolism in age- and ancestry-diverse pediatric populations affected by different types of APS.

Epidemiology of thromboembolic events in children and adolescents with antiphospholipid syndrome: A systematic review with meta-analysis / Epidemiología de los acontecimientos tromboembólicos en niños y adolescentes con síndrome antifosfolípido: una revisión sistemática con metaanálisis

Background and aim: This was a systematic review and meta-analysis of the prevalence of thromboembolic events in children and adolescents with antiphospholipid syndrome (APS). Methods: We searched PubMed, EMBASE and Web of Science to select relevant articles published between 1 January 2000 and 27 February 2022. We used the random-effects meta-analysis to estimate pooled point prevalence rates of thromboembolic events in studies with a minimum sample size of 30. Results: We included five studies reporting data of 336 children and adolescents with primary APS and secondary APS (SAPS). Pooled point prevalence rates of initial general thrombosis, arterial thrombosis, venous thrombosis and stroke in individuals with seropositive APS were 98.2% (95% confidence interval [CI] 87.5–100), 27.6% (95% CI 21.4–34.2), 51.1% (95% CI 38.2–63.9) and 13.4% 95% CI (6.3–22.7), respectively. Pooled point prevalence rates of initial arterial and venous thromboses in children and adolescents with SAPS were 45.7% (95% CI 21.1–71.6) and 29.2% (95% CI 14.8–46), respectively. Conclusion: Arterio-venous thromboembolism is highly frequent in children and adolescents with SAPS. More studies using thrombotic and non-thrombotic APS classification criteria are warranted to better assess the frequency and predictors of thromboembolism in age- and ancestry-diverse pediatric populations affected by different types of APS.(AU)

Antecedentes y objetivo: Se trata de una revisión sistemática y un metaanálisis de la prevalencia de acontecimientos tromboembólicos en niños y adolescentes con síndrome antifosfolípido (SAF). Métodos: Se realizaron búsquedas en PubMed, EMBASE y Web of Science para seleccionar los artículos pertinentes publicados entre el 1 de enero de 2000 y el 27 de febrero de 2022. Se utilizó el metaanálisis de efectos aleatorios para estimar las tasas de prevalencia puntual agrupadas de eventos tromboembólicos en estudios con un tamaño muestral mínimo de 30. Resultados: Se incluyeron cinco estudios con datos de 336 niños y adolescentes con APS primario y APS secundario (SAPS). Las tasas de prevalencia puntual agrupadas de trombosis general inicial, trombosis arterial, trombosis venosa e ictus en individuos con SAF seropositivo fueron de 98,2% (intervalo de confianza [IC] 95%: 87,5-100), 27,6% (IC 95%: 21,4-34,2), 51,1% (IC 95%: 38,2-63,9) y 13,4% (IC 95%: 6,3-22,7), respectivamente. Las tasas de prevalencia puntual agrupadas de trombosis arteriales y venosas iniciales en niños y adolescentes con SAF secundario fueron de 45,7% (IC 95%: 21,1-71,6) y de 29,2% (IC 95%: 14,8-46), respectivamente. Conclusión: La tromboembolia arteriovenosa es muy frecuente en niños y adolescentes con SAF. Se justifica la realización de más estudios que utilicen criterios de clasificación del SCA trombótico y no trombótico para evaluar mejor la frecuencia y los factores predictivos de la tromboembolia en poblaciones pediátricas de edad y ascendencia diversas afectadas por distintos tipos de SCA.(AU)