scholarly journals Hypocomplementemia Associated with Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still’s Disease: 3 Cases: Table 1.

2011 ◽  
Vol 38 (2) ◽  
pp. 396-397 ◽  
Author(s):  
MARK GORELIK ◽  
KATHRYN S. TOROK ◽  
DANIEL A. KIETZ ◽  
RAPHAEL HIRSCH
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

scholarly journals Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance

2020 ◽  
Vol 12 ◽  
pp. 1759720X2097485
Author(s):  
Sofia Ajeganova ◽  
Ann De Becker ◽  
Rik Schots
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Standard Dosage ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.

scholarly journals Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

2019 ◽  
Vol 40 (1) ◽  
pp. 145-152 ◽  
Author(s):  
Ken Goda ◽  
Tsuneaki Kenzaka ◽  
Masahiko Hoshijima ◽  
Akihiro Yachie ◽  
Hozuka Akita
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Yamaguchi Criteria ◽  
Adult Onset Still's Disease ◽  
Cytokine Profiling ◽  
Activation Syndrome

Abstract Adult-onset Still’s disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.

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