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

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

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

The Journal of Rheumatology ◽

10.3899/jrheum.140288 ◽

2015 ◽

Vol 42 (4) ◽

pp. 712-722 ◽

Cited By ~ 51

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.

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