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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doaj-82ef2f54138a4e9cb1ca8e711f3f02f92021-06-04T04:28:28ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2021-06-01810.3389/fmed.2021.681875681875Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic AspectsAltynay Abdirakhmanova0Vitaliy Sazonov1Zaure Mukusheva2Maykesh Assylbekova3Diyora Abdukhakimova4Dimitri Poddighe5Dimitri Poddighe6Department of Medicine, Nazarbayev University School of Medicine (NUSOM), Nur-Sultan, KazakhstanDepartment of Biomedical Sciences, Nazarbayev University School of Medicine (NUSOM), Nur-Sultan, KazakhstanProgram of Pediatric Rheumatology, Department of Pediatrics, National Research Center for Maternal and Child Health, University Medical Center, Nur-Sultan, KazakhstanProgram of Pediatric Rheumatology, Department of Pediatrics, National Research Center for Maternal and Child Health, University Medical Center, Nur-Sultan, KazakhstanDepartment of Medicine, Nazarbayev University School of Medicine (NUSOM), Nur-Sultan, KazakhstanDepartment of Medicine, Nazarbayev University School of Medicine (NUSOM), Nur-Sultan, KazakhstanDepartment of Pediatrics, National Research Center for Maternal and Child Health, University Medical Center, Nur-Sultan, KazakhstanMacrophage 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.https://www.frontiersin.org/articles/10.3389/fmed.2021.681875/fullMacrophage Activation Syndromepediatric Systemic Lupus Erythematosusdiagnostic criteriaferritinhyper-ferritinemia |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Altynay Abdirakhmanova Vitaliy Sazonov Zaure Mukusheva Maykesh Assylbekova Diyora Abdukhakimova Dimitri Poddighe Dimitri Poddighe |
spellingShingle |
Altynay Abdirakhmanova Vitaliy Sazonov Zaure Mukusheva Maykesh Assylbekova Diyora Abdukhakimova Dimitri Poddighe Dimitri Poddighe Macrophage Activation Syndrome in Pediatric Systemic Lupus Erythematosus: A Systematic Review of the Diagnostic Aspects Frontiers in Medicine Macrophage Activation Syndrome pediatric Systemic Lupus Erythematosus diagnostic criteria ferritin hyper-ferritinemia |
author_facet |
Altynay Abdirakhmanova Vitaliy Sazonov Zaure Mukusheva Maykesh Assylbekova Diyora Abdukhakimova Dimitri Poddighe Dimitri Poddighe |
author_sort |
Altynay Abdirakhmanova |
title |
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_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_sort |
macrophage activation syndrome in pediatric systemic lupus erythematosus: a systematic review of the diagnostic aspects |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Medicine |
issn |
2296-858X |
publishDate |
2021-06-01 |
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. |
topic |
Macrophage Activation Syndrome pediatric Systemic Lupus Erythematosus diagnostic criteria ferritin hyper-ferritinemia |
url |
https://www.frontiersin.org/articles/10.3389/fmed.2021.681875/full |
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