Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis under Treatment with Tocilizumab

2015 ◽  
Vol 42 (4) ◽  
pp. 712-722 ◽  
Author(s):  
Shumpei Yokota ◽  
Yasuhiko Itoh ◽  
Tomohiro Morio ◽  
Naokata Sumitomo ◽  
Kaori Daimaru ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Clinical Laboratory ◽  
Laboratory Data ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Manifestations ◽  
Safety Evaluation ◽  
Laboratory Findings ◽  
Activation Syndrome

Objective.To identify macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA) undergoing tocilizumab (TCZ) treatment, and to confirm laboratory marker changes and responses to treatment in patients with MAS receiving TCZ.Methods.In Japan, 394 patients with sJIA were registered in an all-patient registry surveillance of TCZ as of January 15, 2012. TCZ (8 mg/kg) was administered every 2 weeks to patients with sJIA. MAS, hemophagocytic lymphohistiocytosis, or Epstein-Barr virus–associated hemophagocytic syndrome (EB-VAHS) was reported in 23 of these patients (25 events). The Safety Evaluation Committee of Tocilizumab for JIA reviewed these cases and clinically evaluated the data and laboratory findings using their own therapeutic experience. Events were categorized into 4 groups: definitive MAS, probable MAS, EB-VAHS, and non-MAS.Results.The committee’s review revealed 3 events of definitive MAS in 3 patients, 12 events of probable MAS in 11 patients, 2 events of EB-VAHS in 2 patients, and 8 events of non-MAS in 8 patients. There were 2 patients who developed 2 events: 2 events in 1 patient were classified into definitive MAS and probable MAS, and 2 events in another patient were classified into probable MAS. In patients with definitive or probable MAS, common clinical manifestations and laboratory findings of MAS were observed. Changes in laboratory data observed in patients with EB-VAHS were similar to those observed in patients with MAS.Conclusion.These results suggest that the clinical/laboratory features in the course of MAS appear to be similar among patients regardless of whether TCZ is administered. Similarities in the pathophysiological background of MAS and EB-VAHS were also suggested.

An International Consensus Survey of Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

2011 ◽  
Vol 38 (4) ◽  
pp. 764-768 ◽  
Author(s):  
SERGIO DAVÌ ◽  
ALESSANDRO CONSOLARO ◽  
DINARA GUSEINOVA ◽  
ANGELA PISTORIO ◽  
NICOLINO RUPERTO ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Diagnostic Criteria ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Liver Enzymes ◽  
Clinical Laboratory ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Consensus Formation ◽  
International Consensus ◽  
Activation Syndrome

Objective.To identify candidate diagnostic criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) using international consensus formation through a Delphi questionnaire survey.Methods.A questionnaire listing 28 clinical, laboratory, and histopathologic features of MAS elicited by literature review was sent to 505 pediatric rheumatologists worldwide. Respondents were asked to select the 10 features that they felt were most important and useful in the diagnosis of MAS, and to order the 10 selected features by assigning the number 10 to the most important, and ending with 1 as the least important.Results.The response rate was 46% (232 physicians from 47 countries). The items selected by more than 50% of respondents were, in order of frequency, falling platelet count, hyperferritinemia, evidence of macrophage hemophagocytosis in the bone marrow, increased liver enzymes, falling leukocyte count, persistent continuous fever ≥ 38°C, falling erythrocyte sedimentation rate, hypofibrinogenemia, and hypertriglyceridemia.Conclusion.Our process led to identification of features that were felt to be most important as candidate diagnostic criteria for MAS by a large sample of international pediatric rheumatologists.

scholarly journals Macrophage activation syndrome in an inadequately treated patient with systemic onset juvenile idiopathic arthritis

1970 ◽  
Vol 7 (4) ◽  
pp. 411-413
Author(s):  
M Juneja ◽  
R Jain ◽  
D Mishra
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Treated Patient ◽  
Clinical Manifestations ◽  
Altered Mental Status ◽  
Female Child ◽  
Laboratory Findings ◽  
Systemic Onset ◽  
Activation Syndrome

Macrophage activation syndrome is a rare and potentially life threatening complication of childhood rheumatic disorders. It is described most commonly with systemic onset juvenile idiopathic arthritis (soJIA). The major clinical manifestations are non-remitting fever, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, altered mental status and rash and may mimic a flare of soJIA. The characteristic laboratory findings are leucopenia, thrombocytopenia and dramatic elevation of urinary β2 microglobulin. Corticosteroids and cyclosporine are the drugs commonly used in its management. Early diagnosis and prompt treatment can be life saving. We report a case of 12 year old female child with inadequately treated systemic onset juvenile idiopathic arthritis who developed fatal macrophage activation syndrome. The diagnosis and management of macrophage activation syndrome are discussed. Key words: Macrophage activation syndrome; systemic onset juvenile idiopathic arthritis; leucopenia; children. DOI: 10.3126/kumj.v7i4.2764 Kathmandu University Medical Journal (2009) Vol.7, No.4 Issue 28, 411-413

Dissecting the Heterogeneity of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

2015 ◽  
Vol 42 (6) ◽  
pp. 994-1001 ◽  
Author(s):  
Francesca Minoia ◽  
Sergio Davì ◽  
AnnaCarin Horne ◽  
Francesca Bovis ◽  
Erkan Demirkaya ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Clinical Laboratory ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Geographic Location ◽  
Outcome Data ◽  
Large Patient ◽  
Attending Physician ◽  
Activation Syndrome

Objective.To seek insights into the heterogeneity of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA) through the analysis of a large patient sample collected in a multinational survey.Methods.International pediatric rheumatologists and hemato-oncologists entered their patient data, collected retrospectively, in a Web-based database. The demographic, clinical, laboratory, histopathologic, therapeutic, and outcome data were analyzed in relation to (1) geographic location of caring hospital, (2) subspecialty of attending physician, (3) demonstration of hemophagocytosis, and (4) severity of clinical course.Results.A total of 362 patients were included by 95 investigators from 33 countries. Demographic, clinical, laboratory, and histopathologic features were comparable among patients seen in diverse geographic areas or by different pediatric specialists. Patients seen in North America were given biologics more frequently. Patients entered by pediatric hemato-oncologists were treated more commonly with biologics and etoposide, whereas patients seen by pediatric rheumatologists more frequently received cyclosporine. Patients with demonstration of hemophagocytosis had shorter duration of sJIA at MAS onset, higher prevalence of hepatosplenomegaly, lower levels of platelets and fibrinogen, and were more frequently administered cyclosporine, intravenous immunoglobulin (IVIG), and etoposide. Patients with severe course were older, had longer duration of sJIA at MAS onset, had more full-blown clinical picture, and were more commonly given cyclosporine, IVIG, and etoposide.Conclusion.The clinical spectrum of MAS is comparable across patients seen in different geographic settings or by diverse pediatric subspecialists. There was a disparity in the therapeutic choices among physicians that underscores the need to establish uniform therapeutic protocols.

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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