Current treatments in macrophage activation syndrome - METAPHOR projec

Current treatments in macrophage activation syndrome - METAPHOR projec

@rashmi

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2 months ago 581

Science #sjia #MacrophageActivation

Current treatments in macrophage activation syndrome - METAPHOR projec

@rashmi2 months ago

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Current treatment in macrophage activation syndrome worldwide: a systematic literature
review …

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Pediatrics, Karolinska University Hospital, Stockholm, Sweden; 18Department of Women’s and
Chi…

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ABSTRACT
Objective. To assess current treatment in macrophage activation syndrome (MAS) worldwi…

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Keywords: macrophage activation syndrome, haemophagocytic syndromes, haemophagocytic
lymphohis…

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INTRODUCTION
Macrophage activation syndrome (MAS) is an hyperinflammatory life-threatening
co…

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paediatric haematologists from the Histiocyte Society. In this context, a systematic literature …

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moderate-high), and the category of evidence (CoE) was attributed as per EULAR standardized
op…

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All studies mentioned the use of GCs and information were available for 1054 MAS patients
(829…

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etoposide24. Five sJIA-MAS patients were successfully treated with CsA without modification of
…

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to SLE/MCTD was treated for 72 hours without any other medication, but eventually died from
m…

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increasing serious events and infection rate. In the trial 1 patient received emapalumab togeth…

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In our SLR only one study reporting JAK-i was specifically focused on MAS38. In this paper,
a…

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methotrexate, tocilizumab and canakinumab, who was successfully treated with emapalumab as
br…

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Eleven studies reported detailed information about KD-related MAS in 58 patients (Table
3). T…

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SLR had the main purpose of uncovering areas in which evidence regarding MAS treatment is still…

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making a reliable evaluation of its efficacy highly biased. However, CsA was confirmed as the m…

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in MAS treatment; however, so far, evidence on MAS is limited to case reports and to mixed sHLH…

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As a consequence, therapeutic approaches to MAS are still extremely variable, with potential
…

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Lilly, Novartis, Novimmune, Pfizer, Sanofi and SOBI; F.M. received consultancy fees from SOBI a…

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REFERENCES
1. Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, et al. 2016 Classific…

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patients with macrophage activation syndrome complicating systemic juvenile idiopathic
arthri…

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15. Chi Y, Liu R, Zhou ZX, Shi XD, Ding YC, Li JG. Ruxolitinib treatment permits lower cumulati…

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23. Yu TY, Lu MY, Lin KH, Chang HH, Chou SW, Lin DT, et al. Outcomes and prognostic factors
a…

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30. Sönmez HE, Demir S, Bilginer Y, Özen S. Anakinra treatment in macrophage activation
syndr…

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38. He T, Xia Y, Luo Y, Yang J. JAK inhibitors in systemic juvenile idiopathic arthritis. Front…

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46. Borgia RE, Gerstein M, Levy DM, Silverman ED, Hiraki LT. Features, Treatment, and
Outcome…

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53. Yasin S, Solomon K, Canna SW, Girard-Guyonvarc'h C, Gabay C, Schiffrin E, et al. IL-18 as
…

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61. Gokce M, Bilginer Y, Besbas N, Ozaltin F, Cetin M, Gumruk F, et al. Hematological features …

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69. Rivera-Rodriguez L, Pardo-Díaz E, Moreno-Espinosa S, Scheffler-Mendoza S, Ruiz-Ontiveros
…

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TABLES AND FIGURES
Figure 1. Flowchart for the systematic literature review, including detaile…

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Table 1. Data available on patients with MAS treated with anakinra
First author,
year (ref)
Ty…

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16 sHLH (3
malignancies)
SLE/MCTD: 2-
48mg/kg/day (latter as
continuous IV infusion).
Charle…

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Rossano M,
2023 (32)
Retrospective
case series
14 MAS (6 sJIA, 3 SLE, 2
JDM, 3 unknown) 3 s…

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Table 2. Treatment data available on patients with SLE-associated MAS.
First
author,
year (ref…

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Eloseily EM,
2020 (28)
Retrospective
case series US 5 NA
5/5 ANK. Concomitant treatment repor…

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Table 3. Treatment data available on patients with KD-associated MAS.
First author,
year (ref)
…

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ANK: anakinra; ASA: acetylsalicylic acid; CAA: coronary artery aneurism; CoE: category of evidence;…

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Flowchart of the systematic literature review, including detailed exclusion criteria, and results o…

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Lukjanoviˇca K,
2023 (36) Medicina
Single-centre
retrospective
case series
10 sJIA-MAS Latv…

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who continued ANK together with
emapalumab
Shimizu M,
2023 (19)
Int J Rheum
Dis.
Multicent…

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Rhee S, 2023
(70) Children
Single-centre
retrospective
case series
4 KD-MAS Korea 4/4 2nd I…

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arthritis; SLE: systemic lupus erythematosus; TAC: tacrolimus; TCZ: tocilizumab; TMA: thrombotic mi…

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Review of treatments trends for MAS in 2024

Current treatments in macrophage activation syndrome - METAPHOR projec

@rashmi

Science

#sjia #MacrophageActivation

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