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Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)

A prolonged fever in a child can be due to a range of causes including infectious, autoimmune, malignant, or genetic in etiology. In our report, we present the case of a previously healthy three-year-old female diagnosed with macrophage activation syndrome (MAS) due to complications of systemic juve...

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Autores principales: Cortes, Marianne, Nudelman, Brian G, Rouse, Megan J, Frost, Maria D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611171/
https://www.ncbi.nlm.nih.gov/pubmed/37900539
http://dx.doi.org/10.7759/cureus.46083
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author Cortes, Marianne
Nudelman, Brian G
Rouse, Megan J
Frost, Maria D
author_facet Cortes, Marianne
Nudelman, Brian G
Rouse, Megan J
Frost, Maria D
author_sort Cortes, Marianne
collection PubMed
description A prolonged fever in a child can be due to a range of causes including infectious, autoimmune, malignant, or genetic in etiology. In our report, we present the case of a previously healthy three-year-old female diagnosed with macrophage activation syndrome (MAS) due to complications of systemic juvenile arthritis (sJIA). MAS is considered a secondary subtype of hemophagocytic lymphohistiocytosis (HLH), a rare and life-threatening group of syndromes characterized by overstimulation of the immune system leading to systemic inflammation. Through our case, we wanted to bring awareness to this uncommon group of diseases as well as discuss the importance of differentiating between its subtypes. While HLH and MAS have similar clinical presentations, the treatment regimen for each is distinct. Moreover, further research should be conducted to create standardized criteria and treatment guidelines that are evidence-based in order to properly manage these patients.
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spelling pubmed-106111712023-10-28 Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS) Cortes, Marianne Nudelman, Brian G Rouse, Megan J Frost, Maria D Cureus Pediatrics A prolonged fever in a child can be due to a range of causes including infectious, autoimmune, malignant, or genetic in etiology. In our report, we present the case of a previously healthy three-year-old female diagnosed with macrophage activation syndrome (MAS) due to complications of systemic juvenile arthritis (sJIA). MAS is considered a secondary subtype of hemophagocytic lymphohistiocytosis (HLH), a rare and life-threatening group of syndromes characterized by overstimulation of the immune system leading to systemic inflammation. Through our case, we wanted to bring awareness to this uncommon group of diseases as well as discuss the importance of differentiating between its subtypes. While HLH and MAS have similar clinical presentations, the treatment regimen for each is distinct. Moreover, further research should be conducted to create standardized criteria and treatment guidelines that are evidence-based in order to properly manage these patients. Cureus 2023-09-27 /pmc/articles/PMC10611171/ /pubmed/37900539 http://dx.doi.org/10.7759/cureus.46083 Text en Copyright © 2023, Cortes et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pediatrics
Cortes, Marianne
Nudelman, Brian G
Rouse, Megan J
Frost, Maria D
Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title_full Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title_fullStr Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title_full_unstemmed Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title_short Prolonged Fever in a Pediatric Patient: A Case of Systemic Juvenile Idiopathic Arthritis (sJIA) Complicated by Macrophage Activation Syndrome (MAS)
title_sort prolonged fever in a pediatric patient: a case of systemic juvenile idiopathic arthritis (sjia) complicated by macrophage activation syndrome (mas)
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10611171/
https://www.ncbi.nlm.nih.gov/pubmed/37900539
http://dx.doi.org/10.7759/cureus.46083
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