Severe Juvenile-Onset Systemic Lupus Erythematosus: A Case Series-Based Review and Update.

Juvenile-onset systemic lupus erythematosus (jSLE) is a multisystemic disease diagnosed in young patients based on the clinical criteria of the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). The importance of this condition lies in its greater aggressiveness compared with lupus diagnosed during adulthood (aSLE). Management, which is based on supportive care and immunosuppressive drugs, aims to reduce the overall disease activity and to prevent exacerbation. Sometimes the onset is accompanied by life-threatening clinical conditions. In this paper, we introduce three recent cases of jSLE that required admission to the Pediatric Intensive Care Unit (PICU) of a Spanish pediatric hospital. This manuscript aims to review some of the main complications associated with jSLE, such as diffuse alveolar hemorrhage, cerebral vasculitis, or an antiphospholipid syndrome; these are life-threatening conditions but they have a chance of favorable prognosis if treated early and aggressively.

New multivariable prediction model PEdiatric SEpsis recognition and stratification (PESERS score) shows excellent discriminatory capacity.

AIM: To develop a quantitative predictive scoring model for the early recognition and assessment of paediatric sepsis. METHODS: Prospective observational study including emergency department and in-hospital febrile patients under 18 years. Sepsis diagnose (Goldstein 2005 definitions) was the main outcome. Variables associated with the outcome were included in a multivariable analysis. Cut-off points, odds ratio and coefficients for the variables kept after the multivariable analysis were identified. The score was obtained from the coefficients, The AUC was obtained from ROC-analysis, and internal validation was performed using k-fold cross-validation. RESULTS: The analysis included 210 patients. 45 variables were evaluated and the bivariate analysis identified 24 variables associated with the outcome. After the multivariable regression, 11 variables were kept and the score was obtained. The model yielded an excellent AUC of 0.886 (95% CI 0.845-0.927), p < 0.001 for sepsis recognition. With a cut-off value of 5 for the score, we obtained a sensitivity of 98%, specificity of 76.7%, positive predictive value of 87.9% and negative predictive value of 93.3%. CONCLUSION: The proposed scoring model for paediatric sepsis showed adequate discriminatory capacity and sufficient accuracy, which is of great clinical significance in detecting sepsis early and predicting its severity. Nevertheless external validation is needed before clinical use.

Randomized trial: inflammatory response to corticoids versus placebo in moderate and severe bronchiolitis. COTHEB study

ABSTRACT Introduction: The use of corticosteroids has been shown not to improve the prognosis of patients with bronchiolitis, but it could be assumed that steroids will reduce inflammation. Objective: This study aimed to assess whether corticoid therapy influenced the inflammatory and clinical response of critically ill infants. Methods: Prospective, randomized, double blind placebo-controlled trial of glucocorticoids (GCT) in infants of less than 12 months with severe or moderate bronchiolitis. Patients were randomized to receive systemic corticoid therapy (low dose for 7 days) or placebo. The main outcomes were: a) levels of lymphocyte subsets; b) levels of IL-2, IL-12, and IFNγ as pro-inflammatory factors, and c) levels of IL-4 and IL-10 as anti-inflammatory response. Secondary outcomes related with the clinical response were also analyzed. Results: 97 patients were randomized. Evolution of lymphocyte subsets was similar in both groups. Pro-inflammatory interleukins and interferon decreased, but without differences. Anti-inflammatory interleukins showed a significant decrease from baseline to the end of the study, and IL-10 values were significantly lower (p = 0.046) in the GCT group [1.82 pg/ml (1.2-3.5)] vs non-GCT [4 pg/ml (1.5-6.3)]. GCT group showed a lower time of mechanical ventilation and of hospitalization, but without statistically significant differences. No cases of severe adverse reaction to steroids were detected. Conclusions: Administration of systemic GCT did not modify the inflammatory nor the clinical response of patients with severe bronchiolitis, except for IL-10 levels that were significantly lower in the GCT group. This can open a line of investigation about the relation of IL-10 and response to bronchiolitis.

RESUMEN Introducción: Se ha demostrado que los glucocorticoides no mejoran el pronóstico de pacientes con bronquiolitis, pero se podría suponer que reducen la inflamación. Objetivo: Evaluar si los glucocorticoides influyen en la respuesta inflamatoria y clínica de los lactantes críticos. Métodos: Ensayo prospectivo, aleatorizado, doble ciego, controlado con placebo en lactantes < 12 meses con bronquiolitis grave y administración de glucocorticoides sistémicos (dosis bajas 7 días). Se examinaron: a) concentraciones de subconjuntos de linfocitos; b) concentraciones de IL-2, IL-12 e IFNγ como factores proinflamatorios, y c) concentraciones de IL-4 e IL-10 como respuesta antiinflamatoria. También se analizaron los resultados relacionados con la respuesta clínica. Resultados: Se aleatorizaron 97 pacientes. La evolución de los subconjuntos de linfocitos fue similar en ambos grupos. Disminuyeron las interleucinas proinflamatorias y el interferón, pero sin diferencias. Las interleucinas antiinflamatorias mostraron una disminución significativa desde el inicio hasta el final del estudio, y los valores de IL-10 fueron significativamente más bajos (p= 0,046) en el grupo de glucocorticoides [1,82 pg/ml (1,2-3,5)] frente a los no glucocorticoides [4 pg/ml (1,5 - 6,3)]. El grupo glucocorticoides mostró menor tiempo de ventilación mecánica y de hospitalización, pero sin diferencias significativas. No se detectaron reacciones adversas graves a glucocorticoides. Conclusiones: La administración de glucocorticoides sistémicos no modificó la respuesta inflamatoria ni clínica de los pacientes con bronquiolitis severa, excepto las concentraciones de IL-10 que fueron significativamente menores en el grupo de glucocorticoides. Esto puede abrir una línea de investigación sobre la relación de IL-10 y la respuesta a la bronquiolitis.