scholarly journals Macrophage activation syndrome complicating early course of adult-onset Still’s disease

2020 ◽  
pp. 83-83
Author(s):  
Ksenija Bozic ◽  
Marija Elez ◽  
Branislava Glisic
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Early Recognition ◽  
Successful Outcome ◽  
Unknown Etiology ◽  
High Dose ◽  
Adult Onset ◽  
Early Course ◽  
Elevated Liver Enzymes ◽  
Activation Syndrome

Introduction. Adult-onset Still?s disease is a rare inflammatory disorder of unknown etiology. It can be complicated by macrophage activation syndrome, potentially life-treatening condition. While macrophage activation syndrome and adult-onset Still?s disease shared similar features, early recognition is very difficult in clinical praxis. Case outline. We report a young woman, which illness was presented suddenly, with spiking fever, sore throat, myalgia, arthralgia and maculopapular rash. In suspicion of sepsis, she received antibiotics, despite no evidence of infection. After two weeks, her condition worsened, which has been followed by cytopenia, elevated liver enzymes and high serum levels of ferritin. She was diagnosed as macrophage activation syndrome in early course of adult-onset Still?s disease. She was treated with high dose corticosteroids and cyclosporine A and recovered completely. Conclusion. Macrophage activation syndrome can occur at the beginning of adult-onset Still?s disease. Early recognition and timely administration of immunosuppressive drugs are important for successful outcome in this condition.

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.

UnMASking the diagnosis: Acute severe necrotizing pancreatitis in the setting of systemic lupus erythematosus complicated by macrophage activation syndrome

2018 ◽  
Vol 5 (4) ◽  
pp. 1
Author(s):  
Cyrus Ashraf Askin ◽  
Jerome Craig Edelson ◽  
Guy Smith Dooley ◽  
Amy Nicole Stratton
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Acute Pancreatitis ◽  
Inflammatory Response ◽  
Lupus Erythematosus ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Necrotizing Pancreatitis ◽  
Adult Onset ◽  
Systemic Lupus ◽  
Activation Syndrome

Systemic lupus erythematosus (SLE) is a common rheumatologic condition with known GI involvement. Acute pancreatitis (AP) is a rare GI complication of SLE and is typically associated with increased disease activity. Macrophage activation syndrome (MAS) is an unusual, hyper-inflammatory response to a rheumatologic stimulus characterized by hyperferritinemia, pancytopenia, thermal dysregulation and multi-organ dysfunction. MAS, more commonly seen in children, has been reported to complicate both adult onset SLE and AP. We present a case of necrotizing AP secondary to an SLE flare complicated by MAS in an adult patient successfully treated with anakinra.

scholarly journals High-dose anakinra as treatment for macrophage activation syndrome caused by refractory Kawasaki disease in an infant

2019 ◽  
Vol 12 (8) ◽  
pp. e229708 ◽  
Author(s):  
Marie Lind-Holst ◽  
Ulla Birgitte Hartling ◽  
Anne Estmann Christensen
Keyword(s):  
Pericardial Effusion ◽  
Kawasaki Disease ◽  
Coronary Arteries ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Additional Treatment ◽  
High Dose ◽  
Acute Phase Reactants ◽  
High Dose Methylprednisolone ◽  
Activation Syndrome

We report a 12-week-old boy presenting with incomplete refractory Kawasaki disease (KD) complicated with macrophage activation syndrome (MAS). The infant presented with cerebral irritability, pain, tachypnoea and vomiting for 10 days. He did not fulfil any of the classic diagnostic criteria for KD. Pericardial effusion on echocardiography in addition to severe dilatation of the coronary arteries in combination with leucocytosis and raised acute phase reactants led to the diagnosis of incomplete KD. Treatment with intravenous immunoglobulin and aspirin was initiated but without any response. The condition was subsequently refractory to additional treatment with infliximab and high-dose methylprednisolone. His condition worsened, fulfilling the criteria for MAS. High-dose anakinra was initiated, and remission of the inflammation was achieved.

Sign in / Sign up

Export Citation Format

Share Document