Are Children With Kawasaki Disease and Prolonged Fever at Risk for Macrophage Activation Syndrome?

PEDIATRICS ◽  
2003 ◽  
Vol 112 (6) ◽  
pp. e495-e497 ◽  
Author(s):  
A. Muise ◽  
S. E. Tallett ◽  
E. D. Silverman
Keyword(s):  
At Risk ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Prolonged Fever ◽  
Activation Syndrome

Macrophage Activation Syndrome as the Extreme Form of Kawasaki Disease

2010 ◽  
Vol 17 (2) ◽  
pp. 177 ◽  
Author(s):  
Hyoun-Jin Park ◽  
Yoon-Jeong Cho ◽  
E-Young Bae ◽  
Ui-Yoon Choi ◽  
Soo-Young Lee ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Extreme Form ◽  
Activation Syndrome

scholarly journals AB1044 CYTOKINE PROFILE OF MACROPHAGE ACTIVATION SYNDROME ASSOCIATED WITH KAWASAKI DISEASE

2019 ◽  
Author(s):  
Naoto Sakumura ◽  
Masaki Shimizu ◽  
Asumi Jinkawa ◽  
Hitoshi Irabu ◽  
Maiko Takakuwa ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Cytokine Profile ◽  
Activation Syndrome

scholarly journals High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

2019 ◽  
Vol 12 (8) ◽  
pp. e229708 ◽  
Author(s):  
Marie Lind-Holst ◽  
Ulla Birgitte Hartling ◽  
Anne Estmann Christensen
Keyword(s):  
Pericardial Effusion ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Additional Treatment ◽  
High Dose ◽  
Acute Phase Reactants ◽  
High Dose Methylprednisolone ◽  
Activation Syndrome

We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

Should refractory Kawasaki disease be considered occult macrophage activation syndrome?

2017 ◽  
Vol 46 (4) ◽  
pp. e17 ◽  
Author(s):  
Ui-Yoon Choi ◽  
Seung-Beom Han ◽  
Soo-Young Lee ◽  
Dae-Chul Jeong
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Activation Syndrome

Macrophage activation syndrome in children with Kawasaki disease: an experience from a tertiary care hospital in northwest India

Rheumatology ◽  
2020 ◽  
Author(s):  
Rakesh Kumar Pilania ◽  
Ankur Kumar Jindal ◽  
Nameirakpam Johnson ◽  
Ashwini Prithvi ◽  
Pandiarajan Vignesh ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Tertiary Care ◽  
Clinical Signs ◽  
Oral Prednisolone ◽  
Care Hospital ◽  
Coronary Artery Abnormalities ◽  
Activation Syndrome

Abstract Objectives To carry out a review of clinical characteristics, laboratory profiles, management and outcomes of patients with Kawasaki disease (KD) and macrophage activation syndrome (MAS). Methods Medical records of patients treated for KD and MAS between January 1994 and December 2019 were reviewed. Patient demographics, clinical signs, laboratory values, coronary artery abnormalities, treatments and outcomes of patients with KD and MAS were recorded. We also performed a review published studies on the subject. Results Of the 950 cases with KD, 12 (1.3%; 10 boys, 2 girls) were diagnosed with MAS. The median age at diagnosis was 4 years (range 9 months–7.5 years). The median interval between onset of fever and diagnosis of KD was 11 days (range 6–30). Thrombocytopenia was seen in 11 patients. The median pro-brain natriuretic peptide value was 2101 pg/ml (range 164–75 911). Coronary artery abnormalities were seen in 5 (41.7%) patients; 2 had dilatation of the left main coronary artery (LMCA), 1 had dilatation of both the LMCA and right coronary artery (RCA), 1 had dilatation of the RCA and 1 had bright coronary arteries. All patients received IVIG as first-line therapy for KD. MAS was treated with i.v. methylprednisolone pulses followed by tapering doses of oral prednisolone. Additional therapy included i.v. infliximab (n = 4), second-dose IVIG (n = 1) and oral ciclosporin (n = 1). Conclusion MAS is an unusual and underrecognized complication of KD. In our cohort of 950 patients with KD, 1.3% had developed MAS. KD with MAS is associated with an increased propensity towards development of coronary artery abnormalities.

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