Faculty Opinions recommendation of Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial.

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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Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients

Rheumatology ◽

10.1093/rheumatology/keq218 ◽

2010 ◽

Vol 50 (2) ◽

pp. 417-419 ◽

Cited By ~ 166

Author(s):

P. M. Miettunen ◽

A. Narendran ◽

A. Jayanthan ◽

E. M. Behrens ◽

R. Q. Cron

Keyword(s):

Rheumatic Disease ◽

Immunosuppressive Therapy ◽

Successful Treatment ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Interleukin 1 ◽

Case Series ◽

Activation Syndrome

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Macrophage Activation Syndrome Associated with Adult-Onset Still’s Disease Successfully Treated with Anakinra

Case Reports in Rheumatology ◽

10.1155/2016/3717392 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 3

Author(s):

Aswini Kumar ◽

Hiroshi Kato

Keyword(s):

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Interleukin 1 ◽

Pulse Therapy ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Adult Onset Still's Disease ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still’s disease (Still’s disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still’s disease, whether it is therapeutic for MAS associated with Still’s disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation. Striking hyperferritinemia was noted without evidence of infection, malignancy, or hemophagocytosis on bone marrow biopsy. NK cells were undetectable in the peripheral blood, whereas soluble IL-2 receptor was elevated. His multiorgan disease resolved in association with methylprednisolone pulse therapy, Anakinra, and a tapering course of prednisone. This case reinforces the notion that Still’s disease is inherently poised to manifest MAS as one of the clinical phenotypes by shedding light on the role of IL-1 underlying both Still’s disease and related MAS.

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Higher-dose canakinumab therapy for refractory macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: two case reports

Pediatrician (St Petersburg) ◽

10.17816/ped5414-19 ◽

2014 ◽

Vol 5 (4) ◽

pp. 14-19 ◽

Cited By ~ 1

Author(s):

Mikhail Mikhaylovich Kostik ◽

Tatyana Serafimovna Likhacheva ◽

Irina Aleksandrovna Chikova ◽

Natal’ya Valer’yevna Buchinskaya ◽

Natal’ya Nikolaevna Abramova ◽

...

Keyword(s):

Juvenile Idiopathic Arthritis ◽

Macrophage Activation Syndrome ◽

Macrophage Activation ◽

Case Reports ◽

Interleukin 1 ◽

Systemic Juvenile Idiopathic Arthritis ◽

Fatal Complication ◽

Cytokine Inhibition ◽

Life Threatening ◽

Activation Syndrome

Macrophage activation syndrome (MAS) is a life-threatening, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) appears in non-remitted fever, cytopenia, coagulopathy, liver and CNS dysfunctions. Triggers of MAS could be disease activity, infections and medications. Known IL-1 is the key cytokine in pathogenesis of MAS and SJIA, and disease flare associated with increased amounts of different cytokines, especially IL-1β. Many cases of MAS are medically-refractory to traditional doses of cytokine inhibition and may require increased dosing of biologic cytokine blockade. Interleukin-1 (IL-1) is typically a key cytokine in the pathogenesis of sJIA and associated MAS. When MAS occurs in the setting of sJIA treated with IL-1 inhibitors, then increased dosing of IL-1 blockers may be beneficial. This has been shown for anakinra, an IL-1 receptor antagonist, but this drug is currently not available worldwide. Another IL-1 blocker, canakinumbab (CKB), is a monoclonal antibody that blocks IL-1β, but does not also block IL-1α like anakinra. Herein, we describe 2 sJIA patients who developed MAS on standard doses of CKB (4 mg/kg). Both patients received an increased dose of CKB: 150 mg (7.5 and 12.5 mg/kg, respectively) with rapid and complete resolution of MAS. Later the CKB doses was tapered to normal regimen. No side effects or adverse events were noticed during usage of increased CKB doses. Increased dosing of CKB should be considered for CKB-treated sJIA patients who develop MAS on standard dosing.

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