Complicated systemic JIA with macrophage activation syndrome and pulmonary hypertension responsive to a anti IL-1: case report

Abstract Background Subcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis. Recent reports suggest anakinra can be a valuable addition to the treatment of COVID-19 associated cytokine storm syndrome and the related multisystem inflammatory syndrome (MIS-C) in children. Herein, we describe our experience with intravenously administered anakinra. Findings 19 Patients (9 male) received intravenous (IV) anakinra for treatment of macrophage activation syndrome (MAS) secondary to systemic lupus erythematosus (SLE), systemic JIA (SJIA) or secondary hemophagocytic lymphohistiocytosis (sHLH). In most cases the general trend of the fibrinogen, ferritin, AST, and platelet count (Ravelli criteria) improved after initiation of IV anakinra. There were no reports of anaphylaxis or reactions associated with administration of IV anakinra. Conclusion Intravenous administration of anakinra is an important therapeutic option for critically ill patients with MAS/HLH. It is also beneficial for those with thrombocytopenia, subcutaneous edema, neurological dysfunction, or very young, hospitalized patients who need multiple painful subcutaneous injections.

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Case report of a lupus patient with flare and symptomatic cardiac tamponade and macrophage activation syndrome: A biochemical diagnostic perspective

Medical Journal of Dr D Y Patil Vidyapeeth ◽

10.4103/mjdrdypu.mjdrdypu_195_20 ◽

2022 ◽

Vol 15 (1) ◽

pp. 101

Author(s):

Kapil Bhatia ◽

Amandeep Singh ◽

Pratibha Misra ◽

Bhasker Mukherjee ◽

VivekN Ambade

Keyword(s):

Case Report ◽

Cardiac Tamponade ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Lupus Patient ◽

Activation Syndrome

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Macrophage activation syndrome – A physician’s nightmare: A case report on life threatening complication of adult-onset Still’s disease

Journal of the Postgraduate Institute of Medicine ◽

10.4038/jpgim.8315 ◽

2021 ◽

Vol 8 (2) ◽

pp. 147

Author(s):

Sanchayan Thanancheyan ◽

Nisanthan Selvaratnam ◽

Sivagamasundary Uthayakumaran ◽

Narani Aravinthan ◽

Thaneswary Sooriyakumar ◽

...

Keyword(s):

Case Report ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Life Threatening ◽

Life Threatening Complication ◽

Adult Onset Still's Disease ◽

Activation Syndrome

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P034 Macrophage activation syndrome in Juvenile Idiopathic Arthritis

Rheumatology ◽

10.1093/rheumatology/keab722.026 ◽

2021 ◽

Vol 60 (Supplement_5) ◽

Author(s):

Rahma Guedri ◽

Glaimeriem ◽

Zohra Fitouri ◽

Saayda Ben Becher ◽

Bechir Hamza

Keyword(s):

Biological Treatment ◽

Pediatric Rheumatology ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Systemic Jia ◽

Rheumatology Unit ◽

The Mean ◽

And Biological Markers ◽

Activation Syndrome

Abstract Objectives To describe the epidemiological, clinical-biological, and therapeutic characteristics of children with systemic JIA complicated with a macrophage activation syndrome (MAS). Methods It was a retrospective study, including children with JIA followed at the pediatric rheumatology unit of the children's hospital in Tunis for 22 years (January 1999 to December 2020), and presented MAS during their follow-up. Results We included 40 patients with JIA. Nineteen children (47.5%) presented MAS during the disease. They are 11 boys and 8 girls, with a sex ratio of 1.3. The mean age was 5.31 years (range: 0.66–10.83 years). The circumstances of the occurrence were variable. MAS was inaugural in 10 patients. Three of our patients presented MAS twice. MAS was definitive with clinical and biological markers in 12 cases and only biological in 7 cases. Seventeen of our patients were treated with intravenous Corticosteroids. Seven Childs among them received, in combination, Immunoglobulins (IG). We prescribed a biological treatment in 3 patients. We have mourned only one death. Conclusion MAS is a severe complication of JIA and is the leading cause of death.

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