Related Experiment Video
Updated: Dec 21, 2025
Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
Published on: December 26, 2013
Macrophage activation syndrome in children with Kawasaki disease: diagnostic and therapeutic approaches
Seung Beom Han1, Soo-Young Lee2
1Department of Pediatrics, Daejeon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 64 Daeheung-ro, Jung-gu, Daejeon, 34943, Republic of Korea.
Background:
Macrophage activation syndrome (MAS) is a rare, life-threatening complication of Kawasaki disease (KD). Early recognition and treatment of MAS are very important, but sometimes it is difficult to distinguish MAS from a severe form of KD.
Data Sources:
A PubMed search was performed in Clinical Queries using the key terms "macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis (HLH)" and "Kawasaki disease".
Results:
KD patients with MAS show high intravenous immunoglobulin (IVIG) resistance and coronary complications. Mortality is also as high as MAS in other diseases. Persistent fever greater than 10 days is highly associated with development of MAS in KD. Splenomegaly is observed in more than two-thirds of KD patients with MAS. Thrombocytopenia is often the earliest laboratory finding of MAS. Hyperferritinemia is highly specific and sensitive for detecting MAS in KD; so, ferritin levels should be checked if there are unexplained clinical exacerbations in KD patients. Given the under-recognition of MAS in KD, it is prudent to consider resistant KD as occult/subclinical MAS. Many KD patients with MAS have good outcomes on immune modulators. However, if KD patients fulfill the HLH-2004 diagnostic criteria, they may undergo longer and more intensive treatment than needed.
Conclusions:
The possible existence of MAS should be taken into account when a KD patient shows persistent fever, splenomegaly, thrombocytopenia, hyperferritinemia, or IVIG resistance. The under-diagnosis of MAS in patients with KD is an important issue to be addressed. Therapeutically, however, there is a possibility of over-treatment of MAS in patients with KD.
Insights
Macrophage activation syndrome (MAS) is a rare complication of Kawasaki disease (KD). Early recognition of MAS in KD patients is crucial, as it can be mistaken for severe KD, leading to potential over-treatment.
Area of Science:
- Pediatrics
- Rheumatology
- Immunology
Background:
- Macrophage activation syndrome (MAS) is a life-threatening complication of Kawasaki disease (KD).
- Distinguishing MAS from severe KD is clinically challenging.
- Early diagnosis and treatment of MAS in KD are critical.
Purpose of the Study:
- To review the key features and diagnostic challenges of MAS in KD.
- To highlight the importance of recognizing MAS in KD patients.
- To discuss therapeutic considerations for MAS in KD.
Main Methods:
- A comprehensive PubMed search was conducted using specific keywords.
- The search focused on studies combining "macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis (HLH)" and "Kawasaki disease".
Main Results:
- KD patients with MAS often exhibit IVIG resistance and coronary complications.
- Persistent fever (>10 days), splenomegaly, and thrombocytopenia are common indicators of MAS in KD.
- Hyperferritinemia is a highly sensitive and specific marker for MAS in KD; consider it in unexplained exacerbations.
- Under-recognition of MAS in KD is noted, with resistant KD potentially representing subclinical MAS.
- While many KD patients with MAS respond to immune modulators, adherence to HLH-2004 criteria may lead to over-treatment.
Conclusions:
- MAS should be suspected in KD patients with persistent fever, splenomegaly, thrombocytopenia, hyperferritinemia, or IVIG resistance.
- Under-diagnosis of MAS in KD is a significant issue requiring attention.
- There is a risk of over-treatment for MAS in KD patients, necessitating careful diagnostic evaluation.
