Objective To develop an early prediction nomogram for identifying the risk of macrophage activation syndrome (MAS) in children with Kawasaki disease (KD). Methods This study retrospectively collected clinical and laboratory data from KD children with MAS (KD-MAS, n = 38) between January 2019 and May 2024. Classic KD patients (KD, n = 338) and intravenous immunoglobulin (IVIG)-resistant KD patients (n = 291) were selected as control groups. Results Children with KD-MAS had longer fever time, more frequent hepatomegaly and splenomegaly, and pericardial effusion, higher serum ferritin levels and lower platelet and fibrinogen levels compared with KD group and IVIG-resistant KD group. Notably, significantly increased serum interleukin-10 and interferon-γ (IFN-γ) levels were observed in children with KD-MAS. Longer fever duration (> 10 days, OR, 9.89; 95% CI 4.35–22.49), decreased platelet count (≤ 181 × 10^9/L, OR, 27.85; 95% CI 12.33–62.97), higher serum ferritin levels (> 684ug/L, OR, 112.73; 95% CI 40.74–311.88), low fibrinogen levels (≤ 3.6 g/L, OR, 33.47; 95% CI 13.16–85.13), and high IFN-γ levels (≥ 30 pg/mL, OR, 10.73; 95% CI 4.64–24.87) were independent predictors of MAS. These nomograms incorporating serum IFN-γ plus clinical variables were comparable to the classical model based on serum ferritin, platelet, and fibrinogen, underscoring the importance of serum IFN-γ as a key indicator in the early recognition of MAS in children with KD or IVIG-resistant KD. Conclusion The prediction nomograms suggest that, within this single-center retrospective cohort, serum IFN-γ serves as a promising biomarker and plays a valuable role in the early recognition of MAS in children with KD or IVIG-resistant KD. Its combined application with prolonged fever and elevated ferritin can improve predictive efficacy.<Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec colname="c1" colnum="1" /> <colspec colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> Key Points • Serum IFN-γ serves as a promising biomarker, and in conjunction with key clinical indicators, it plays a valuable role in the early recognition of MAS in children with KD or IVIG-resistant KD. • Prolonged fever (> 10 days), low platelets (≤ 181 × 10⁹/L), high ferritin (> 684 μg/L), and low fibrinogen (≤ 3.6 g/L) are significant clinical indicators of MAS. • A nomogram incorporating IFN-γ and clinical variables performs comparably to classic models, highlighting its utility in early MAS detection. </entry> </row> </tbody> </tgroup> </Table>

Prediction nomogram of serum interferon-γ enable early identification of macrophage activation syndrome in children with Kawasaki disease

期刊论文
发表日期：

2025年11月12日

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Yana Wang,
Yini Wang,
Jia Wang,
Yun Zhou,
Luhong Zhu,
Keying Wang,
Liping Teng,
Bin Hu,
Meiping Lu,
Xuefeng Xu

摘要

Objective

To develop an early prediction nomogram for identifying the risk of macrophage activation syndrome (MAS) in children with Kawasaki disease (KD).

Methods

This study retrospectively collected clinical and laboratory data from KD children with MAS (KD-MAS, n = 38) between January 2019 and May 2024. Classic KD patients (KD, n = 338) and intravenous immunoglobulin (IVIG)-resistant KD patients (n = 291) were selected as control groups.

Results

Children with KD-MAS had longer fever time, more frequent hepatomegaly and splenomegaly, and pericardial effusion, higher serum ferritin levels and lower platelet and fibrinogen levels compared with KD group and IVIG-resistant KD group. Notably, significantly increased serum interleukin-10 and interferon-γ (IFN-γ) levels were observed in children with KD-MAS. Longer fever duration (> 10 days, OR, 9.89; 95% CI 4.35–22.49), decreased platelet count (≤ 181 × 10^9/L, OR, 27.85; 95% CI 12.33–62.97), higher serum ferritin levels (> 684ug/L, OR, 112.73; 95% CI 40.74–311.88), low fibrinogen levels (≤ 3.6 g/L, OR, 33.47; 95% CI 13.16–85.13), and high IFN-γ levels (≥ 30 pg/mL, OR, 10.73; 95% CI 4.64–24.87) were independent predictors of MAS. These nomograms incorporating serum IFN-γ plus clinical variables were comparable to the classical model based on serum ferritin, platelet, and fibrinogen, underscoring the importance of serum IFN-γ as a key indicator in the early recognition of MAS in children with KD or IVIG-resistant KD.

Conclusion

The prediction nomograms suggest that, within this single-center retrospective cohort, serum IFN-γ serves as a promising biomarker and plays a valuable role in the early recognition of MAS in children with KD or IVIG-resistant KD. Its combined application with prolonged fever and elevated ferritin can improve predictive efficacy.

Key Points

• Serum IFN-γ serves as a promising biomarker, and in conjunction with key clinical indicators, it plays a valuable role in the early recognition of MAS in children with KD or IVIG-resistant KD.

• Prolonged fever (> 10 days), low platelets (≤ 181 × 10⁹/L), high ferritin (> 684 μg/L), and low fibrinogen (≤ 3.6 g/L) are significant clinical indicators of MAS.

• A nomogram incorporating IFN-γ and clinical variables performs comparably to classic models, highlighting its utility in early MAS detection.