Outcome of Children With Systemic Rheumatologic and Autoinflammatory Diseases Admitted to a Pediatric Intensive Care Unit: A Retrospective Cohort Study With Development of a Prognostic Risk-stratification Tool.

Nearly two-thirds (62.2%) of patients experienced their first disease manifestation as a life-threatening crisis.

Backward stepwise logistic regression identified 3 predictors that were combined, with equal weighting, into the exploratory PHV Score (PRISM-HLH-Vasoactive): Pediatric Risk of Mortality III (Pediatric Risk of Mortality III) at 24 hours >20, HLH/MAS diagnosis, and requirement for ≥2 vasoactive agents.