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.

scholarly journals Successful treatment of macrophage activation syndrome complicating adult-onset Still’s disease with tocilizumab: a case report

2017 ◽  
Vol 17 (2) ◽  
pp. 64-68
Author(s):  
Ho So ◽  
Tak-Lung Wong ◽  
Hin Ting Pang ◽  
Weng Nga Lao ◽  
Man-Lung Yip
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Initial Control ◽  
Adult Onset Still's Disease ◽  
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.

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 An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation

2014 ◽  
Vol 2014 ◽  
pp. 1-7
Author(s):  
Rajaie Namas ◽  
Naveen Nannapaneni ◽  
Malini Venkatram ◽  
Gulcin Altinok ◽  
Miriam Levine ◽  
...  
Keyword(s):  
Hemophagocytic Syndrome ◽  
Adult Onset Still’S Disease ◽  
Normocytic Anemia ◽  
High Dose ◽  
African American Female ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Adult Onset Still's Disease

Case. A 34-year-old African-American female with a history of adult-onset Still’s disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizureen routevia ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital’s burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired.Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD.

Sign in / Sign up

Export Citation Format

Share Document