Macrophage Activation Syndrome: A Potentially Fatal Complication of Kawasaki Disease

2015 ◽  
Vol 30 (2) ◽  
pp. 178-180 ◽  
Author(s):  
Kallol Bose
Keyword(s):  
Kawasaki Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Fatal Complication ◽  
Activation Syndrome

Reaching the Threshold: A Multilayer Pathogenesis of Macrophage Activation Syndrome

2013 ◽  
Vol 40 (6) ◽  
pp. 761-767 ◽  
Author(s):  
Raffaele Strippoli ◽  
Ivan Caiello ◽  
Fabrizio De Benedetti
Keyword(s):  
Mouse Models ◽  
Inflammatory Cytokines ◽  
Genetic Background ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Molecular Mechanisms ◽  
Fatal Complication ◽  
Pathogenic Factors ◽  
Laboratory Features ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic diseases. The condition is considered part of secondary hemophagocytic lymphohistiocytoses (HLH). There are similarities in genetic background, pathogenesis, and clinical and laboratory features with primary HLH (p-HLH). We describe findings in mouse models of secondary HLH, comparing them with models of p-HLH and the cellular and molecular mechanisms involved, and relate them to recent findings in patients with secondary HLH. A multilayer model is presented in which background inflammation, infections, and genetics all contribute in different proportions and in several ways. Once the “threshold” has been reached, inflammatory cytokines are the final effectors, independent of the interplay between different upstream pathogenic factors.

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.

scholarly journals 66. Macrophage activation syndrome: a fatal complication in a patient with SLE

2018 ◽  
Vol 2 (suppl_1) ◽  
Author(s):  
Vishal Kakkar ◽  
Nikolaos Dadiras ◽  
Bela Szebenyi ◽  
Tim Gillott ◽  
Amir Alvi
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Fatal Complication ◽  
Activation Syndrome
Sign in / Sign up

Export Citation Format

Share Document