Clinical Features, Treatment, and Outcome of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A Multinational, Multicenter Study of 362 Patients

2014 ◽  
Vol 66 (11) ◽  
pp. 3160-3169 ◽  
Author(s):  
Francesca Minoia ◽  
Sergio Davì ◽  
AnnaCarin Horne ◽  
Erkan Demirkaya ◽  
Francesca Bovis ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Clinical Features ◽  
Multicenter Study ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Activation Syndrome

scholarly journals Tocilizumab modifies clinical and laboratory features of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Masaki Shimizu ◽  
Mao Mizuta ◽  
Nami Okamoto ◽  
Takahiro Yasumi ◽  
Naomi Iwata ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Clinical Features ◽  
Early Treatment ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Classification Criteria ◽  
Real Patient ◽  
Laboratory Features ◽  
Activation Syndrome

Abstract Background This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ. Methods A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS. Results Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients. Conclusion TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIA patients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.

Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

2005 ◽  
Vol 146 (5) ◽  
pp. 598-604 ◽  
Author(s):  
Angelo Ravelli ◽  
Silvia Magni-Manzoni ◽  
Angela Pistorio ◽  
Cristina Besana ◽  
Tiziana Foti ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Activation Syndrome
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