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© 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.

Abstract

Background: Macrophage activation syndrome (MAS) is a hyperinflammatory and potentially fatal complication associated with rheumatologic disorders. In Kawasaki disease (KD), MAS is a rare and poorly described condition, making its differentiation from a severe, treatment-resistant presentation of KD particularly challenging. Objective: We aimed to describe MAS in KD by analyzing its epidemiological, clinical, and laboratory characteristics, complications, therapeutic strategies, and outcomes. Methods: A comprehensive literature review of PubMed, Embase, Scopus, and Cochrane Library was conducted to identify English-language studies on KD complicated by MAS, including case reports and case series, until 15 November 2024. Results: A total of 176 pediatric patients (60 females; median age 4 years, range 0.13–17) from 48 articles were included. MAS occurred after or simultaneously with KD diagnosis in 174/176 cases (99%). Common features included fever (100%), splenomegaly (49.4%), and hyperferritinemia (98.2%). Cardiac involvement was reported in 37% of children. The HLH-2004 criteria were met in 63% of cases, while the 2016 Ravelli criteria for MAS complicating systemic juvenile idiopathic arthritis were met in 94%. Treatment included additional doses of IVIG (36.2%), GCs (82.8%), cyclosporine A (28.7%), and biologics (13.8%), with complete MAS resolution in 93% of cases. Conclusions: MAS in KD is a rare but severe complication, with overlapping features that make its differentiation from severe and resistant KD challenging. Persistent fever despite initial IVIG administration, along with splenomegaly and hyperferritinemia, emerge as key warning signs. Ravelli criteria provide stronger diagnostic support compared to the HLH-2004 criteria. Moreover, MAS is associated with increased cardiac involvement.

Details

Title
Macrophage Activation Syndrome in Kawasaki Disease: Insights from a Systematic Literature Review on Diagnosis, Clinical Features, and Treatment
Author
Inguscio, Giulia 1 ; Romano, Stefano 1   VIAFID ORCID Logo  ; Mastrolia, Maria Vincenza 2 ; Simonini, Gabriele 2   VIAFID ORCID Logo  ; Giani, Teresa 2   VIAFID ORCID Logo 

 Pediatric Department, School of Sciences of Human Health, University of Florence, 50121 Florence, Italy 
 Rheumatology Unit, ERN ReCONNET Center, AOU Meyer Children’s Hospital IRCCS, 50139 Florence, Italy[email protected] (G.S.) 
First page
349
Publication year
2025
Publication date
2025
Publisher
MDPI AG
e-ISSN
22279067
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
3181405048
Copyright
© 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.