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Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease
Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still’s disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes...
Autores principales: | , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8745834/ https://www.ncbi.nlm.nih.gov/pubmed/35011947 http://dx.doi.org/10.3390/jcm11010206 |
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author | Javaux, Clément El-Jammal, Thomas Neau, Pierre-Antoine Fournier, Nicolas Gerfaud-Valentin, Mathieu Perard, Laurent Fouillet-Desjonqueres, Marine Le Scanff, Julie Vignot, Emmanuelle Durupt, Stéphane Hot, Arnaud Belot, Alexandre Durieu, Isabelle Henry, Thomas Sève, Pascal Jamilloux, Yvan |
author_facet | Javaux, Clément El-Jammal, Thomas Neau, Pierre-Antoine Fournier, Nicolas Gerfaud-Valentin, Mathieu Perard, Laurent Fouillet-Desjonqueres, Marine Le Scanff, Julie Vignot, Emmanuelle Durupt, Stéphane Hot, Arnaud Belot, Alexandre Durieu, Isabelle Henry, Thomas Sève, Pascal Jamilloux, Yvan |
author_sort | Javaux, Clément |
collection | PubMed |
description | Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still’s disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14–11.2; p = 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08–15.3; p = 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 μg/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (p < 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS. |
format | Online Article Text |
id | pubmed-8745834 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-87458342022-01-11 Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease Javaux, Clément El-Jammal, Thomas Neau, Pierre-Antoine Fournier, Nicolas Gerfaud-Valentin, Mathieu Perard, Laurent Fouillet-Desjonqueres, Marine Le Scanff, Julie Vignot, Emmanuelle Durupt, Stéphane Hot, Arnaud Belot, Alexandre Durieu, Isabelle Henry, Thomas Sève, Pascal Jamilloux, Yvan J Clin Med Article Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still’s disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14–11.2; p = 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08–15.3; p = 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 μg/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (p < 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS. MDPI 2021-12-31 /pmc/articles/PMC8745834/ /pubmed/35011947 http://dx.doi.org/10.3390/jcm11010206 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Javaux, Clément El-Jammal, Thomas Neau, Pierre-Antoine Fournier, Nicolas Gerfaud-Valentin, Mathieu Perard, Laurent Fouillet-Desjonqueres, Marine Le Scanff, Julie Vignot, Emmanuelle Durupt, Stéphane Hot, Arnaud Belot, Alexandre Durieu, Isabelle Henry, Thomas Sève, Pascal Jamilloux, Yvan Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title | Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title_full | Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title_fullStr | Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title_full_unstemmed | Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title_short | Detection and Prediction of Macrophage Activation Syndrome in Still’s Disease |
title_sort | detection and prediction of macrophage activation syndrome in still’s disease |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8745834/ https://www.ncbi.nlm.nih.gov/pubmed/35011947 http://dx.doi.org/10.3390/jcm11010206 |
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