FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4311273050> ?p ?o ?g. }

• W4311273050 abstract "ABSTRACT Objectives Macrophage activation syndrome (MAS) is a severe, life-threatening complication of systemic juvenile idiopathic arthritis (sJIA) and adult onset Still’s disease (AOSD). The objective of this study was to confirm the adequacy of an emapalumab dosing regimen in relation to interferon-γ (IFNγ) activity by assessing efficacy and safety. The efficacy outcome was MAS remission by week 8, based on clinical and laboratory criteria. Methods We studied emapalumab, a human anti–IFNγ antibody, administered with background glucocorticoids, in a prospective open-label, single-group trial involving patients who had MAS secondary to sJIA or AOSD and had previously failed high-dose glucocorticoids. The study foresaw 4-week treatment that could be shortened or prolonged based on investigator’s assessment of response. Patients could enter a long-term (12 months) follow-up study. Results Fourteen patients received emapalumab. All patients completed the trial, entered the long-term follow-up and were alive at the end of the follow-up. The investigated dosing regimen, based on an initial loading dose followed by maintenance doses, was appropriate, as shown by rapid neutralisation of IFNγ activity, demonstrated by prompt decrease in serum C-X-C motif chemokine ligand 9 (CXCL9) levels. By week 8, remission of MAS was achieved in 13 of the 14 patients at a median time of 25 days. Viral infections and positive viral tests were observed during the trial and during the long-term follow-up. Conclusions Neutralisation of IFNγ with emapalumab was efficacious in inducing remission of MAS secondary to sJIA or AOSD in patients who had failed high-dose glucocorticoids. KEY MESSAGES What is already known on this topic? Macrophage activation syndrome (MAS) is a severe and potentially life-threatening complication of systemic juvenile idiopathic arthritis and adult-onset Still’s disease. There are no therapeutic options that have been prospectively investigated in MAS. Data in animal models and ex vivo data from humans with MAS led to the hypothesis that interferon-γ (IFNγ) has a pathogenic role in MAS. What does this study add? This open-label multicentre trial using emapalumab, an anti–IFNγ antibody, in patients who have failed to respond to high-dose glucocorticoids, demonstrates that IFNγ has a pathogenic role in MAS and that its neutralisation leads to MAS remission. How might this affect research, clinical practice or policy? The results of this study show that neutralisation of IFNγ with emapalumab is a therapeutic option for patients with severe MAS who have failed standard of care with high-dose glucocorticoids." @default.
• W4311273050 created "2022-12-25" @default.
• W4311273050 creator A5002868596 @default.
• W4311273050 creator A5004658434 @default.
• W4311273050 creator A5010084443 @default.
• W4311273050 creator A5020926202 @default.
• W4311273050 creator A5025765870 @default.
• W4311273050 creator A5031743563 @default.
• W4311273050 creator A5054849094 @default.
• W4311273050 creator A5055904789 @default.
• W4311273050 creator A5059134648 @default.
• W4311273050 creator A5059983393 @default.
• W4311273050 creator A5065701007 @default.
• W4311273050 creator A5068254685 @default.
• W4311273050 creator A5076916358 @default.
• W4311273050 creator A5079738832 @default.
• W4311273050 creator A5085179292 @default.
• W4311273050 creator A5089949725 @default.
• W4311273050 creator A5090531557 @default.
• W4311273050 date "2022-12-13" @default.
• W4311273050 modified "2023-10-15" @default.
• W4311273050 title "Efficacy and safety of emapalumab in macrophage activation syndrome" @default.
• W4311273050 cites W1505537322 @default.
• W4311273050 cites W1980376496 @default.
• W4311273050 cites W1990180387 @default.
• W4311273050 cites W2094736478 @default.
• W4311273050 cites W2188441859 @default.
• W4311273050 cites W2265918137 @default.
• W4311273050 cites W2304277276 @default.
• W4311273050 cites W2444247510 @default.
• W4311273050 cites W2608377891 @default.
• W4311273050 cites W2742469269 @default.
• W4311273050 cites W2782451473 @default.
• W4311273050 cites W2784026193 @default.
• W4311273050 cites W2908053584 @default.
• W4311273050 cites W2933607365 @default.
• W4311273050 cites W2969456052 @default.
• W4311273050 cites W2973124663 @default.
• W4311273050 cites W2979892428 @default.
• W4311273050 cites W3021363743 @default.
• W4311273050 cites W3021560285 @default.
• W4311273050 cites W3044576993 @default.
• W4311273050 cites W3107820052 @default.
• W4311273050 cites W3112059656 @default.
• W4311273050 cites W3130908087 @default.
• W4311273050 cites W3136896643 @default.
• W4311273050 cites W3138289408 @default.
• W4311273050 cites W3153186428 @default.
• W4311273050 cites W3167694762 @default.
• W4311273050 cites W3167744464 @default.
• W4311273050 cites W3175255495 @default.
• W4311273050 cites W3194400168 @default.
• W4311273050 cites W3197143241 @default.
• W4311273050 cites W3202912135 @default.
• W4311273050 cites W3208854628 @default.
• W4311273050 cites W4225253017 @default.
• W4311273050 cites W4225275535 @default.
• W4311273050 doi "https://doi.org/10.1101/2022.12.12.22283141" @default.
• W4311273050 hasPublicationYear "2022" @default.
• W4311273050 type Work @default.
• W4311273050 citedByCount "0" @default.
• W4311273050 crossrefType "posted-content" @default.
• W4311273050 hasAuthorship W4311273050A5002868596 @default.
• W4311273050 hasAuthorship W4311273050A5004658434 @default.
• W4311273050 hasAuthorship W4311273050A5010084443 @default.
• W4311273050 hasAuthorship W4311273050A5020926202 @default.
• W4311273050 hasAuthorship W4311273050A5025765870 @default.
• W4311273050 hasAuthorship W4311273050A5031743563 @default.
• W4311273050 hasAuthorship W4311273050A5054849094 @default.
• W4311273050 hasAuthorship W4311273050A5055904789 @default.
• W4311273050 hasAuthorship W4311273050A5059134648 @default.
• W4311273050 hasAuthorship W4311273050A5059983393 @default.
• W4311273050 hasAuthorship W4311273050A5065701007 @default.
• W4311273050 hasAuthorship W4311273050A5068254685 @default.
• W4311273050 hasAuthorship W4311273050A5076916358 @default.
• W4311273050 hasAuthorship W4311273050A5079738832 @default.
• W4311273050 hasAuthorship W4311273050A5085179292 @default.
• W4311273050 hasAuthorship W4311273050A5089949725 @default.
• W4311273050 hasAuthorship W4311273050A5090531557 @default.
• W4311273050 hasBestOaLocation W43112730501 @default.
• W4311273050 hasConcept C126322002 @default.
• W4311273050 hasConcept C141071460 @default.
• W4311273050 hasConcept C203014093 @default.
• W4311273050 hasConcept C2777077863 @default.
• W4311273050 hasConcept C2777288759 @default.
• W4311273050 hasConcept C2779613421 @default.
• W4311273050 hasConcept C2781413609 @default.
• W4311273050 hasConcept C71924100 @default.
• W4311273050 hasConcept C81182388 @default.
• W4311273050 hasConceptScore W4311273050C126322002 @default.
• W4311273050 hasConceptScore W4311273050C141071460 @default.
• W4311273050 hasConceptScore W4311273050C203014093 @default.
• W4311273050 hasConceptScore W4311273050C2777077863 @default.
• W4311273050 hasConceptScore W4311273050C2777288759 @default.
• W4311273050 hasConceptScore W4311273050C2779613421 @default.
• W4311273050 hasConceptScore W4311273050C2781413609 @default.
• W4311273050 hasConceptScore W4311273050C71924100 @default.
• W4311273050 hasConceptScore W4311273050C81182388 @default.
• W4311273050 hasLocation W43112730501 @default.
• W4311273050 hasOpenAccess W4311273050 @default.

• next