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Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects

Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging...

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Autores principales: Abdirakhmanova, Altynay, Sazonov, Vitaliy, Mukusheva, Zaure, Assylbekova, Maykesh, Abdukhakimova, Diyora, Poddighe, Dimitri
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8211727/
https://www.ncbi.nlm.nih.gov/pubmed/34150813
http://dx.doi.org/10.3389/fmed.2021.681875
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author Abdirakhmanova, Altynay
Sazonov, Vitaliy
Mukusheva, Zaure
Assylbekova, Maykesh
Abdukhakimova, Diyora
Poddighe, Dimitri
author_facet Abdirakhmanova, Altynay
Sazonov, Vitaliy
Mukusheva, Zaure
Assylbekova, Maykesh
Abdukhakimova, Diyora
Poddighe, Dimitri
author_sort Abdirakhmanova, Altynay
collection PubMed
description Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging. In order to address this issue, we performed a systematic review of the available medical literature, aiming to retrieve all those papers providing diagnostic (clinical/laboratory) data on patients with pSLE-related MAS, in individual or aggregated form. The selected case reports and series provided a pool of 46 patients, accounting for 48 episodes of MAS in total. We re-analyzed these patients in light of the diagnostic criteria for MAS validated in systemic Juvenile Idiopathic Arthritis (sJIA) patients and the preliminary diagnostic criteria for MAS in pSLE, respectively. Five clinical studies were also selected and used to support this analysis. This systematic review confirms that MAS diagnosis in pSLE patients is characterized by several diagnostic challenges, which could lead to delayed diagnosis and/or under-estimation of this complication. Specific criteria should be considered to diagnose MAS in different rheumatic diseases; as regards pSLE, the aforementioned preliminary criteria for MAS in pSLE seem to perform better than the sJIA-related MAS criteria, because of a lower ferritin cut-off.
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spelling pubmed-82117272021-06-19 Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects Abdirakhmanova, Altynay Sazonov, Vitaliy Mukusheva, Zaure Assylbekova, Maykesh Abdukhakimova, Diyora Poddighe, Dimitri Front Med (Lausanne) Medicine Macrophage Activation Syndrome (MAS) is a very severe complication of different rheumatic diseases, including pediatric Systemic Lupus Erythematosus (pSLE). MAS is not considered as a frequent complication of pSLE; however, its occurrence could be under-estimated and the diagnosis can be challenging. In order to address this issue, we performed a systematic review of the available medical literature, aiming to retrieve all those papers providing diagnostic (clinical/laboratory) data on patients with pSLE-related MAS, in individual or aggregated form. The selected case reports and series provided a pool of 46 patients, accounting for 48 episodes of MAS in total. We re-analyzed these patients in light of the diagnostic criteria for MAS validated in systemic Juvenile Idiopathic Arthritis (sJIA) patients and the preliminary diagnostic criteria for MAS in pSLE, respectively. Five clinical studies were also selected and used to support this analysis. This systematic review confirms that MAS diagnosis in pSLE patients is characterized by several diagnostic challenges, which could lead to delayed diagnosis and/or under-estimation of this complication. Specific criteria should be considered to diagnose MAS in different rheumatic diseases; as regards pSLE, the aforementioned preliminary criteria for MAS in pSLE seem to perform better than the sJIA-related MAS criteria, because of a lower ferritin cut-off. Frontiers Media S.A. 2021-06-04 /pmc/articles/PMC8211727/ /pubmed/34150813 http://dx.doi.org/10.3389/fmed.2021.681875 Text en Copyright © 2021 Abdirakhmanova, Sazonov, Mukusheva, Assylbekova, Abdukhakimova and Poddighe. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Medicine
Abdirakhmanova, Altynay
Sazonov, Vitaliy
Mukusheva, Zaure
Assylbekova, Maykesh
Abdukhakimova, Diyora
Poddighe, Dimitri
Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title_full Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title_fullStr Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title_full_unstemmed Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title_short Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects
title_sort macrophage activation syndrome in pediatric systemic lupus erythematosus: a systematic review of the diagnostic aspects
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8211727/
https://www.ncbi.nlm.nih.gov/pubmed/34150813
http://dx.doi.org/10.3389/fmed.2021.681875
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