Enterovirus-Associated HLH: Addition of Anakinra to IVIG and Corticosteroids

Hardman, Simon; Tukur, Gidado; Waruiru, Catherine

doi:10.1007/s10875-019-00639-y

Cited by 6 publications

(3 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Despite its FDA designation, anakinra is used off label to treat a variety of autoinflammatory diseases. Although limited, there are now case reports and series showing both sIJA‐related MAS (19‐22) and other HLH secondary subtypes (22‐27) responding favorably to anakinra. As such, it appears that anakinra may be viewed as an emerging and effective therapy in the treatment of MAS.…”

Section: Introductionmentioning

confidence: 99%

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

Monteagudo

Boothby

Gertner

2020

ACR Open Rheumatology

166

149

View full text Add to dashboard Cite

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.

show abstract

Section: Introductionmentioning

confidence: 99%

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

Monteagudo

Boothby

Gertner

2020

ACR Open Rheumatology

166

149

View full text Add to dashboard Cite

show abstract

“…Anakinra, an interleukin-1-receptor antagonist, has shown e cacy in the treatment of secondary HLH [34] and EV-associated HLH [35]. Moreover, its use has been also reported in a preliminary experience treating pediatric acute fulminant myocarditis, following the presumed central role of IL-1 pathways in pathogenesis [36].…”

Section: Discussionmentioning

confidence: 99%

Clinical course and peculiarities of Parechovirus and Enterovirus central nervous system infections in newborns

Brisca,

Bellini,

Pasquinucci

et al. 2024

Preprint

View full text Add to dashboard Cite

Purpose Parechoviruses (HpEV) and Enteroviruses (EV) infections in children mostly have a mild course but are particularly fearsome in newborns in whom they may cause aseptic meningitis, encephalitis, and myocarditis. Our study aimed to describe the clinical presentations and peculiarities of CNS infection by HpEV and EV in neonates. Methods Single-center retrospective study at Istituto Gaslini, Genoa, Italy. Infants aged ≤30 days with a CSF RTq-PCR positive for EV or HpEV from January 1, 2022, to December 1, 2023, were enrolled. Each patient's record included demographic data, blood and CSF tests, brain MRI, therapies, length of stay, ICU admission, complications, and mortality. The two groups were compared to identify any differences and similarities. Results Twenty-five patients (15 EV and 10 HpEV) with a median age of 15 days were included. EV patients had more comorbidities (p=0.021), more respiratory symptoms (p=0.012), and higher C-reactive protein (CRP) levels on admission (p=0.027), whereas ferritin values were significantly increased in HpEV patients (p=0.001). Eight patients had a pathological brain MRI, equally distributed between the two groups. Three EV patients developed myocarditis and one HpEV necrotizing enterocolitis with HLH-like. No deaths occurred. Conclusions EV and HpEV CNS infections are not easily distinguishable by clinical features. In both cases, brain MRI abnormalities are not uncommon and a severe course of the disease is possible. Hyper-ferritinemia may represent an additional diagnostic clue for HpEV infection and its monitoring is recommended to intercept HLH early and initiate immunomodulatory treatment. Larger studies are needed to confirm our findings.

show abstract

“…The addition of anakinra to intravenous immunoglobulin and corticosteroids was reported to lead to marked clinical improvement in a 13-month-old boy with enterovirus-associated HLH. 3 In terms of pathogenesis, primary and secondary HLH share a common terminal pathway with elevated inflammatory markers driven by T cells leading to a cytokine storm. HLH is driven by a defect in natural killer cells and cytotoxic T lymphocytes, which is permanent in primary HLH and transient in secondary HLH.…”

Section: Discussionmentioning

confidence: 99%

Enterovirus-associated hemophagocytic lymphohistiocytosis with multiorgan failure

Ramani

Sudhakaran

Rahimi

et al. 2020

Baylor University Medical Center Proceedings

View full text Add to dashboard Cite

show abstract

Enterovirus-Associated HLH: Addition of Anakinra to IVIG and Corticosteroids

Cited by 6 publications

References 4 publications

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

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

Clinical course and peculiarities of Parechovirus and Enterovirus central nervous system infections in newborns

Enterovirus-associated hemophagocytic lymphohistiocytosis with multiorgan failure

Contact Info

Product

Resources

About