Outcome of Children With Systemic Rheumatologic and Autoinflammatory Diseases Admitted to a Pediatric Intensive Care...

Area of Science:

• Pediatric critical care medicine
• Rheumatology
• Immunology

Background:

• Children with systemic rheumatologic diseases (SRD) and autoinflammatory diseases can experience severe, life-threatening deterioration requiring pediatric intensive care unit (PICU) admission.
• A significant proportion of these children present with their first disease manifestation as a critical event.

Purpose of the Study:

• To describe the clinical presentations and outcomes of children with SRD admitted to the PICU.
• To develop an exploratory bedside risk-stratification tool to aid in assessing severity for these specific patient populations.

Main Methods:

• Retrospective observational cohort study.
• Development of a prognostic model using backward stepwise logistic regression.
• Propensity-score matching for secondary descriptive analysis.
• Single academic PICU data from January 2005 to December 2015.

Main Results:

• Hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) was linked to the majority of deaths (83.3%) despite lower admission rates (20%).
• The exploratory PHV Score (PRISM-HLH-Vasoactive), combining PRISM-III, HLH/MAS diagnosis, and vasoactive agent use, showed promising discrimination (apparent AUC 0.91).
• At the optimal cutoff (≥2), the PHV Score demonstrated high sensitivity (83.3%) and specificity (91.2%) in the derivation cohort.

Conclusions:

• HLH/MAS is a major driver of mortality in critically ill children with SRD.
• The PHV Score is a potential bedside tool for refining risk stratification in this population.
• Prospective, multicenter external validation is required for the PHV Score.