THU0403 Scd163 in Adult Onset Still's Disease: A Biomarker for Macrophage Activation Related to Hyperferritinemia:

2014 ◽  
Vol 73 (Suppl 2) ◽  
pp. 321.1-321
Author(s):  
S. Colafrancesco ◽  
R. Priori ◽  
E. Astorri ◽  
C. Perricone ◽  
N. Agmon-Levin ◽  
...  
Keyword(s):  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

scholarly journals Successful Treatment with Intravenous Cyclophosphamide for Refractory Adult-Onset Still’s Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Yoshika Tsuji ◽  
Nozomi Iwanaga ◽  
Anna Adachi ◽  
Kinuyo Tsunozaki ◽  
Yasumori Izumi ◽  
...  
Keyword(s):  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Intravenous Cyclophosphamide ◽  
Still's Disease ◽  
Female Case ◽  
High Doses ◽  
Adult Onset Still's Disease

We report a 64-year-old female case of intractable adult-onset Still’s disease (AOSD). Initial high-dose steroid therapy combined with cyclosporin A was ineffective against macrophage-activation syndrome (MAS), which was accompanied by the systemic type of AOSD. Treatment for MAS with intravenous cyclophosphamide resulted in remission of AOSD and a reduction in the high doses of steroids. Efficacy of biologics against MAS in AOSD is unclear. Cyclophosphamide, a conventional cytotoxic agent, should be considered as one of the therapeutic options for refractory types of AOSD with MAS.

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.

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