Des traitements comme l'anakinra, un inhibiteur de l'IL-1, sont en cours d'évaluation.
AnakinraInhibiteurs de l'IL-1
#5
Quelle est la durée du traitement ?
La durée du traitement varie, mais peut durer plusieurs semaines à plusieurs mois selon la réponse.
Durée du traitementRéponse au traitement
Complications
5
#1
Quelles sont les complications possibles du syndrome ?
Les complications incluent l'insuffisance hépatique, l'insuffisance rénale et le choc.
Insuffisance hépatiqueInsuffisance rénale
#2
Le syndrome peut-il être fatal ?
Oui, sans traitement approprié, le syndrome peut entraîner la mort en raison de défaillances organiques.
MortDéfaillance organique
#3
Y a-t-il des risques à long terme ?
Des risques à long terme incluent des séquelles organiques et un risque accru de récidive.
SéquellesRécidive
#4
Comment gérer les complications ?
La gestion des complications nécessite une approche multidisciplinaire et un suivi régulier.
Gestion des complicationsSuivi médical
#5
Les complications affectent-elles la qualité de vie ?
Oui, les complications peuvent significativement affecter la qualité de vie des patients.
Qualité de vieComplications
Facteurs de risque
5
#1
Quels sont les facteurs de risque du syndrome ?
Les facteurs incluent des maladies auto-immunes, des infections virales et des antécédents familiaux.
Maladies auto-immunesAntécédents familiaux
#2
L'âge influence-t-il le risque ?
Oui, le syndrome est plus fréquent chez les enfants et les jeunes adultes.
PédiatrieJeunes adultes
#3
Les infections peuvent-elles déclencher le syndrome ?
Oui, certaines infections virales, comme la grippe, peuvent déclencher le syndrome.
Infections viralesGrippe
#4
Y a-t-il un lien avec des traitements immunosuppresseurs ?
Oui, les traitements immunosuppresseurs peuvent augmenter le risque d'activation macrophagique.
ImmunosuppresseursTraitements
#5
Le stress peut-il être un facteur de risque ?
Le stress physique ou émotionnel peut exacerber les maladies sous-jacentes et augmenter le risque.
StressMaladies sous-jacentes
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"@type": "Question",
"name": "Les symptômes varient-ils selon l'âge ?",
"position": 9,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les symptômes peuvent être plus sévères chez les enfants et les personnes âgées."
}
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{
"@type": "Question",
"name": "Peut-on avoir des symptômes respiratoires ?",
"position": 10,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des symptômes respiratoires comme la dyspnée peuvent également se manifester dans ce syndrome."
}
},
{
"@type": "Question",
"name": "Peut-on prévenir le syndrome d'activation macrophagique ?",
"position": 11,
"acceptedAnswer": {
"@type": "Answer",
"text": "La prévention est difficile, mais un suivi régulier des maladies sous-jacentes peut aider."
}
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{
"@type": "Question",
"name": "Y a-t-il des mesures préventives spécifiques ?",
"position": 12,
"acceptedAnswer": {
"@type": "Answer",
"text": "Éviter les infections et gérer les maladies auto-immunes peut réduire le risque d'activation."
}
},
{
"@type": "Question",
"name": "Les vaccinations sont-elles recommandées ?",
"position": 13,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les vaccinations peuvent aider à prévenir certaines infections qui déclenchent le syndrome."
}
},
{
"@type": "Question",
"name": "Le mode de vie influence-t-il le risque ?",
"position": 14,
"acceptedAnswer": {
"@type": "Answer",
"text": "Un mode de vie sain peut renforcer le système immunitaire et réduire le risque d'activation."
}
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"@type": "Question",
"name": "Les traitements préventifs existent-ils ?",
"position": 15,
"acceptedAnswer": {
"@type": "Answer",
"text": "Des traitements préventifs peuvent être envisagés pour les patients à haut risque, selon les cas."
}
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{
"@type": "Question",
"name": "Quel est le traitement principal du syndrome ?",
"position": 16,
"acceptedAnswer": {
"@type": "Answer",
"text": "Le traitement principal inclut des corticostéroïdes et des agents immunosuppresseurs."
}
},
{
"@type": "Question",
"name": "Des transfusions sanguines sont-elles nécessaires ?",
"position": 17,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des transfusions peuvent être nécessaires en cas de cytopénies sévères."
}
},
{
"@type": "Question",
"name": "Le traitement est-il le même pour tous les patients ?",
"position": 18,
"acceptedAnswer": {
"@type": "Answer",
"text": "Non, le traitement doit être individualisé selon la gravité et la cause sous-jacente."
}
},
{
"@type": "Question",
"name": "Y a-t-il des traitements expérimentaux ?",
"position": 19,
"acceptedAnswer": {
"@type": "Answer",
"text": "Des traitements comme l'anakinra, un inhibiteur de l'IL-1, sont en cours d'évaluation."
}
},
{
"@type": "Question",
"name": "Quelle est la durée du traitement ?",
"position": 20,
"acceptedAnswer": {
"@type": "Answer",
"text": "La durée du traitement varie, mais peut durer plusieurs semaines à plusieurs mois selon la réponse."
}
},
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"@type": "Question",
"name": "Quelles sont les complications possibles du syndrome ?",
"position": 21,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les complications incluent l'insuffisance hépatique, l'insuffisance rénale et le choc."
}
},
{
"@type": "Question",
"name": "Le syndrome peut-il être fatal ?",
"position": 22,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, sans traitement approprié, le syndrome peut entraîner la mort en raison de défaillances organiques."
}
},
{
"@type": "Question",
"name": "Y a-t-il des risques à long terme ?",
"position": 23,
"acceptedAnswer": {
"@type": "Answer",
"text": "Des risques à long terme incluent des séquelles organiques et un risque accru de récidive."
}
},
{
"@type": "Question",
"name": "Comment gérer les complications ?",
"position": 24,
"acceptedAnswer": {
"@type": "Answer",
"text": "La gestion des complications nécessite une approche multidisciplinaire et un suivi régulier."
}
},
{
"@type": "Question",
"name": "Les complications affectent-elles la qualité de vie ?",
"position": 25,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les complications peuvent significativement affecter la qualité de vie des patients."
}
},
{
"@type": "Question",
"name": "Quels sont les facteurs de risque du syndrome ?",
"position": 26,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les facteurs incluent des maladies auto-immunes, des infections virales et des antécédents familiaux."
}
},
{
"@type": "Question",
"name": "L'âge influence-t-il le risque ?",
"position": 27,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, le syndrome est plus fréquent chez les enfants et les jeunes adultes."
}
},
{
"@type": "Question",
"name": "Les infections peuvent-elles déclencher le syndrome ?",
"position": 28,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, certaines infections virales, comme la grippe, peuvent déclencher le syndrome."
}
},
{
"@type": "Question",
"name": "Y a-t-il un lien avec des traitements immunosuppresseurs ?",
"position": 29,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les traitements immunosuppresseurs peuvent augmenter le risque d'activation macrophagique."
}
},
{
"@type": "Question",
"name": "Le stress peut-il être un facteur de risque ?",
"position": 30,
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"@type": "Answer",
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Division of Rheumatology, Cincinnati Children' Hospital Medical Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Publications dans "Syndrome d'activation macrophagique" :
Division of Rheumatology, Children's of Alabama and Department of Pediatrics, University of Alabama at Birmingham School of Medicine, Birmingham, AL, USA. rcron@peds.uab.edu.
Publications dans "Syndrome d'activation macrophagique" :
Pediatric Immuno-Hematology and Rheumatology Unit, RAISE Rare Disease Reference Centre, Hopital Universitaire Necker-Enfants Malades, Assistance Publique-Hopitaux de Paris, Paris, France.
Université Paris-Cité, Paris, France.
Publications dans "Syndrome d'activation macrophagique" :
Department of Dermatology, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China. fanghongzy@zju.edu.cn.
Publications dans "Syndrome d'activation macrophagique" :
Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Publications dans "Syndrome d'activation macrophagique" :
Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Publications dans "Syndrome d'activation macrophagique" :
Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.
Publications dans "Syndrome d'activation macrophagique" :
Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. lmdong@tjh.tjmu.edu.cn.
Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China. lmdong@tjh.tjmu.edu.cn.
Publications dans "Syndrome d'activation macrophagique" :
Department of Trauma Surgery, Emergency Surgery & Surgical Critical, Tongji Trauma Center, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. chendeng@tjh.tjmu.edu.cn.
Department of Emergency and Critical Care Medicine, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China. chendeng@tjh.tjmu.edu.cn.
Publications dans "Syndrome d'activation macrophagique" :
Macrophage activation syndrome (MAS), is a severe and fatal complication of various pediatric inflammatory disorders. Kabuki syndrome (KS), mainly caused by lysine methyltransferase 2D (...
BACKGROUNDMacrophage activation syndrome (MAS) is a life-threatening complication of Still's disease (SD) characterized by overt immune cell activation and cytokine storm. We aimed to further understa...
Nowadays, people have relaxed their vigilance against COVID-19 due to its declining infection numbers and attenuated virulence. However, COVID-19 still needs to be concern due to its emerging variants...
Macrophage activation syndrome (MAS) is a rare illness, especially in critically ill adults. The diagnosis of MAS is challenging, requiring the expertise of multiple specialists, and treatments for MA...
We describe the case of a 31-yr-old Vietnamese student who was diagnosed with cutaneous systemic lupus erythematosus (SLE) in November 2020 and was initiated on treatment with low-dose corticosteroids...
Four features of this case merit discussion, including the: 1) infrequent association of SLE with MAS; 2) short interval between SLE diagnosis and critical illness; 3) manifestation of fungal tracheob...
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by the uncontrolled activation of cytotoxic T lymphocytes, NK cells, and macrophages, resulting in an overproduct...
Haemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) has a potentially high mortality rate. Anakinra, an interleukin-1 receptor antagonist, is now recommended early in HLH...
We screened Embase, PubMed, and Medline, including all reports of IV anakinra for HLH or MAS. We extracted age, HLH/MAS trigger, continuous infusion or bolus dosing, and survival....
Twenty-nine case reports/series identified 87 patients (median age 22 years, range 22 months to 84 years), all with sHLH. Amongst identifiable triggers, 43% were systemic infection, 33% rheumatologica...
Despite IV anakinra recipients likely to be critically unwell, this cohort had similar disease triggers and survival compared to large historical cohorts, and enhances awareness of age and trigger-spe...
Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases. The coexistence of MAS and juvenile dermatomyositis (JDM) is not well reported. This...
The patient was a 15-year-old female with JDM, presenting with heliotrope rash, muscle weakness, increased muscle enzyme, anti-nuclear matrix protein 2 (NXP2) antibody, and muscle biopsy consistent wi...
. The coexistence of JDM and MAS is underestimated with increased mortality. Hepatosplenomegaly and increased serum levels of ferritin in patients with JDM should raise clinical suspicion for MAS....
The macrophage activation syndrome (MAS) secondary to systemic lupus erythematosus (SLE) is a severe and life-threatening complication. Early diagnosis of MAS is particularly challenging. In this stud...
We retrospectively collected clinical data from 188 patients with either SLE or the MAS secondary to SLE. 13 significant clinical predictor variables were filtered out using the Least Absolute Shrinka...
Among all the machine learning models, the LR model demonstrates the highest level of performance in internal validation, achieving a ROC-AUC of 0.998, an F1 score of 0.96, and an F2 score of 0.952. T...
Machine learning models can significantly improve the diagnostic accuracy of MAS secondary to SLE, and the diagnostic scorecard model can facilitate personalized probabilistic predictions of disease o...
A 56-year-old woman was treated for rheumatoid arthritis for 17 years with methotrexate (MTX). Night sweats, fever and weight loss made her visit our hospital. Although levofloxacin failed to resolve ...
Macrophage activation syndrome (MAS), an example of secondary hemophagocytic lymphohistiocytosis, is a potentially fatal complication of rheumatic diseases. We aimed to study the clinical and laborato...
Fifty-five patients with rheumatic diseases complicated by MAS were enrolled between January 2017 and December 2022. Clinical and laboratory data were collected before disease onset, at diagnosis, and...
Most (81.8%) instances of MAS occurred during the initial diagnosis of the underlying disease. Compared to the active stage of sJIA, the platelet count, erythrocyte sedimentation rate, and fibrinogen ...
Thrombocytopenia, ferritin levels, the ferritin/erythrocyte sedimentation rate, and elevated aspartate aminotransferase levels can predict the occurrence of MAS in patients with sJIA. Additionally, ou...
