Abstract
BACKGROUND: Systemic immune-mediated diseases can be quite severe in both course and complications, causing multiple organ failure and death. These patients are often required to be hospitalized in an intensive care unit (ICU). AIM: To find early predictors of death in patients with immune-inflammatory diseases hospitalized in the ICU. METHODS: The study included 51 patients (23 boys, 28 girls) with immune-inflammatory diseases, including multisystem inflammatory syndrome associated with coronavirus disease 2019 (n = 18), systemic rheumatic diseases (n = 24), and generalized infections (n = 9) aged from 7 months up to 17 years old, admitted to the ICU of the clinic of Saint Petersburg State Pediatric Medical University in the period from 2007 to 2023. All patients were divided into those with a fatal outcome (n = 13) and those who recovered (n = 38). Macrophage activation syndrome (MAS) was diagnosed by the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation criteria. RESULTS: First-day predictors were white blood cell ≤ 3.1 × 10(9)/L, platelets ≤ 168 × 10(9)/L, diuresis ≤ 1.5 mL/kg/hour, low saturation, K(+) > 4.7 mmol/L, albumin ≤ 30 g/L, creatinine > 74 μmol/L, pH ≤ 7.36, HCO3(-) ≤ 22.2, Glasgow Coma Scale score ≤ 13, Sequential Organ Failure Assessment (SOFA) score > 2, oxygen therapy, mechanical ventilation (MV), fresh frozen plasma transfusions and biological treatment. The third-day predictors were: White blood cell ≤ 4.0 × 10(9)/L, platelets ≤ 63 × 10(9)/L, hemoglobin ≤ 87 g/L, C-reactive protein (CRP) > 129 mg/L, triglycerides > 2.45 mmol/L, albumin ≤ 28 g/L, creatinine > 83.5 μmol/L, pH ≤ 7.38, Glasgow Coma Scale score ≤ 10, SOFA score > 2 and need in MV, intravenous immunoglobulin, and blood transfusion requirements. On the fifth day, the main predictors were CRP > 28 mg/L, triglycerides > 2.3 mmol/L, creatinine > 58 μmol/L, fibrinogen > 3.3 g/L, compliance with the MAS criteria, Glasgow Coma Scale score ≤ 14, SOFA score > 2, and need for MV, vasopressors, and anticoagulant therapy, as well as blood and fresh frozen plasma transfusions. The seventh-day predictors were CRP > 19.1 mg/L, albumin ≤ 35 g/L, total protein ≤ 55 g/L, compliance with the MAS criteria, Glasgow Coma Scale score ≤ 12, SOFA score > 3, and need for MV and biological and anticoagulant therapy. CONCLUSION: Hemaphagocytosis (leukopenia, thrombocytopenia, hyperferritinemia, increased histochemistry score), progressive decline in Glasgow Coma Scale, increasing SOFA scores, and persistent high CRP levels were markers of an unfavorable outcome in patients with immune-mediated inflammatory diseases.