AB1190 Macrophage activation syndrome as the presenting manifestation of systemic juvenile arthritis: Unicenter study of clinical features and outcome in 12 patients

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 705.17-705 ◽  
Author(s):  
R. Russo ◽  
M.M. Katsicas
2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Masaki Shimizu ◽  
Mao Mizuta ◽  
Nami Okamoto ◽  
Takahiro Yasumi ◽  
Naomi Iwata ◽  
...  

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.


Children ◽  
2021 ◽  
Vol 8 (4) ◽  
pp. 269
Author(s):  
Da Eun Roh ◽  
Jung Eun Kwon ◽  
Hee Joung Choi ◽  
Yeo Hyang Kim

We aimed to evaluate the utility of the serum ferritin level as an early screening test of Kawasaki disease with macrophage activation syndrome (KD-MAS). We analyzed the serum ferritin levels on the first day of admission and the clinical progress of patients diagnosed with complete or incomplete KD. Of the 158 patients, 5 were diagnosed with KD-MAS. Conjunctival injection was significantly more frequent in KD group (p = 0.035), although there were no significant differences in other clinical features. On the first day of admission, the serum ferritin level in the KD-MAS group was >500 ng/mL, which was higher than that in the KD group (p = 0.001). In the KD-MAS group, total bilirubin, triglyceride, and lactate dehydrogenase (LDH) were significantly higher, and erythrocyte sedimentation rate (ESR), total protein, albumin, and fibrinogen were significantly lower than the KD group (p < 0.05). Four patients were diagnosed with MAS within 7 days after admission, and 4 (80%) patients with KD-MAS survived. In conclusion, carrying out an early ferritin screening test is important in patients with principal clinical features that may suspect KD. We propose to include ferritin level in the primary laboratory test to differentiate between KD with and without MAS early.


2015 ◽  
Vol 11 (9) ◽  
pp. 1043-1053 ◽  
Author(s):  
Randy Q Cron ◽  
Sergio Davi ◽  
Francesca Minoia ◽  
Angelo Ravelli

1996 ◽  
Vol 129 (5) ◽  
pp. 750-754 ◽  
Author(s):  
Richard Mouy ◽  
Jean-Louis Stephan ◽  
Pascal Pillet ◽  
Elie Haddad ◽  
Philippe Hubert ◽  
...  

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