PReS-FINAL-2041: Macrophage activation syndrome in the children with systemic juvenile idiopathic arthritis during the course of tocilizumab
Е. И. АлексееваР.В. ДенисоваС И ВалиеваТ.М. БзароваK. IsayevaТ. В. СлепцоваEA MitenkoЕ. G. ChistyakovaA. Fetisova
0
Citation
0
Reference
10
Related Paper
Abstract:
Systemic juvenile idiopathic arthritis (sjia) is a chronic inflammatory disease characterized by prolonged systemic and synovial inflammation. Life-threatening complication of sjia is the macrophage activation syndrome (MAS). Tocilizumab (TCZ) is highly effective in children with sjia. However, MAS remains under the treatment of TCZ.Keywords:
Tocilizumab
Macrophage Activation Syndrome
Abstract We report a case of 50-year-old female patient with adult-onset Still’s disease (AOSD) complicated by macrophage-activation syndrome (MAS). After initial control of the disease with high-dose parenteral corticosteroids, tocilizumab (TCZ) therapy aided in maintaining the remission with rapid tapering of steroid dose. TCZ may be useful for MAS complicating AOSD.
Tocilizumab
Macrophage Activation Syndrome
Adult-onset Still's disease
Cite
Citations (0)
Tocilizumab (TCZ) is the first FDA- approved treatment for systemic juvenile idiopathic arthritis (sJIA). We report 3 cases of cytopenias in children with sJIA treated with TCZ. Two of the children who developed significant cytopenias shortly after initiation of TCZ had a history of macrophage activation syndrome. We raise the possibility that patients with a tendency towards MAS have an increased risk of developing cytopenias when treated with tocilizumab.
Tocilizumab
Macrophage Activation Syndrome
Cite
Citations (29)
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.
Tocilizumab
Macrophage Activation Syndrome
Hemophagocytic Lymphohistiocytosis
Cite
Citations (99)
Macrophage activation syndrome (MAS) is a form of secondary hemophagocytic lymphohistiocytosis and is a rapidly progressive, life-threatening complication of adult-onset Still's disease (AOSD). An anti-IL-6 receptor monoclonal antibody, tocilizumab, has shown to be effective in the treatment of AOSD but may precipitate MAS in patients with AOSD. The precise mechanism of MAS developed during anti-cytokine biologic agents remains unknown, but selective inhibition of a subset of pathways could impact other immune signalling pathways and trigger MAS. We herein describe a case of AOSD with the opposite outcomes of tocilizumab therapy, remission and development of MAS, after tocilizumab treatment at the initial flare and the relapse. From the comparison of clinical characteristics and concomitant treatment around the time of starting tocilizumab in both flares, the type and intensity of concomitant immunosuppressive therapy might strongly affect MAS development during tocilizumab therapy.
Tocilizumab
Macrophage Activation Syndrome
Concomitant
Cytokine Release Syndrome
Hemophagocytic Lymphohistiocytosis
Cite
Citations (12)
Tocilizumab
Macrophage Activation Syndrome
Interleukin-6 receptor
Pathogenesis
Cite
Citations (45)
Tocilizumab
Macrophage Activation Syndrome
Cite
Citations (10)
Tocilizumab
Macrophage Activation Syndrome
Interleukin 18
Cite
Citations (120)
We report a 57-year-old female case of intractable adult-onset Still's disease (AOSD). Initial high-dose prednisolone therapy was ineffective, and macrophage-activation syndrome (MAS) manifested after one session of additional tocilizumab therapy. After successful treatment for MAS with lipo-dexamethasone and cyclosporin, tocilizumab therapy aided in the rapid reduction of the therapeutic steroid dose. Tocilizumab may be useful for maintenance therapy for AOSD, although its efficacy is unclear for the highly active phase of the disease.
Tocilizumab
Macrophage Activation Syndrome
Adult-onset Still's disease
Prednisolone
Antirheumatic drugs
Combination therapy
Cytokine Release Syndrome
Cite
Citations (81)
Systemic juvenile idiopathic arthritis (sjia) is a chronic inflammatory disease characterized by prolonged systemic and synovial inflammation. Life-threatening complication of sjia is the macrophage activation syndrome (MAS). Tocilizumab (TCZ) is highly effective in children with sjia. However, MAS remains under the treatment of TCZ.
Tocilizumab
Macrophage Activation Syndrome
Cite
Citations (0)
Background: Nonspecific interstitial pneumonia (NSIP) is a rare pulmonary complication in systemic juvenile idiopathic arthritis (SJIA).Objective: To present a case with NSIP in SJIA Methods: Case report Results: We report the case of a 4-year-old boy with SJIA complicated by macrophage activation syndrome (MAS) refractory to conventional therapy, and who later developed NSIP confirmed by high-resolution computerized tomography of the chest and lung histopathology.The patient received tocilizumab, a monoclonal antibody to interleukin-6 receptor, to control his disease.Data relating to tocilizumab treatment of NSIP in refractory SJIA is limited. Conclusions:The data from this case report suggests that NSIP could be a pulmonary complication of SJIA complicated by MAS that is refractory to conventional therapy.Early initiation of tocilizumab should be considered to achieve disease remission in this pediatric patient population.
Tocilizumab
Macrophage Activation Syndrome
Refractory (planetary science)
Hemophagocytosis
Cite
Citations (8)