Continuous Intravenous Anakinra Infusion to Calm the Cytokine Storm in Macrophage Activation Syndrome

Abstract

Objective The objective of this study was to report the benefit of a therapeutic approach consisting of intravenous (IV) continuous anakinra (recombinant human interleukin‐1 receptor antagonist) infusions in treating severely ill adult patients with secondary hemophagocytic lymphohistiocytosis or macrophage activation syndrome (MAS). Methods A retrospective chart review of five patients treated at Regions Hospital from 2016 to 2019 was conducted. Demographic, clinical, and laboratory characteristics and outcomes were recorded. Results Continuous IV anakinra infusions up to 2400 mg/d resulted in rapid serologic, then clinical response in 4 of 5 severely ill patients who were refractory to all other therapies, including subcutaneous anakinra. Subsequently, 3 of 5 patients have been maintained on anakinra or canakinumab, with no recurrence of MAS. Conclusion Continuous infusion of IV anakinra may result in rapid serologic and subsequent clinical improvement in adult patients with MAS. This method for treating cytokine storm should be considered in the current COVID‐19 pandemic in the subgroup of patients with severe disease who have a cytokine storm presentation.

Continuous intravenous anakinra for treating severe secondary haemophagocytic lymphohistiocytosis/macrophage activation syndrome in critically ill children

“…This strategy enabled curtailing high doses of steroids (otherwise necessary for treating HLH), whilst managing intercurrent infections with appropriate antimicrobials. This highlights the safety of anakinra in sHLH in the presence of infections, as has been noted in previous studies 7–11,19–21,23 . Anakinra infusion was titrated as per our infusion protocol (Supporting Data S1) and weaned with satisfactory clinical progress closely corroborated to laboratory parameters.…”

Early IL ‐1 Inhibition in Still's Disease: A Window of Opportunity for Improving Outcomes

“…Regarding the administration route, although ANK is typically administered SC at a scheduled 100 mg/day, IV administration was employed in this study, before switching to the SC route, only in the patients who are hospitalized most at risk of MAS or during MAS. The IV route, previously reported in children with MAS and Multisystem Inflammatory Syndrome in Children, 24–26 gained traction during the SARS‐CoV‐2 pandemic, when ANK was approved 27 for COVID‐19; we observed benefits of IV ANK in severe Still's disease cases, in terms of clinical and laboratory parameters, including those triggered by COVID‐19 vaccination, 28,29 before transitioning to SC administration.…”

Anakinra in Paediatric Rheumatology and Periodic Fever Clinics: Is the Higher Dose Safe?

“…Full responders to anakinra had shorter previous disease duration than partial responders, an observation that is in agreement with the recent report on predictors of anakinra response ( 43 ). Anakinra use in MAS or other secondary HLH was recently reported by several groups ( 16 – 18 , 33 , 34 , 44 , 45 ). Despite different anakinra dosing, all of them reported its beneficial effect in majority of patients, although comparisons are difficult mainly due to variable concomitant corticosteroid use.…”