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Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in...

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Autores principales: Ajeganova, Sofia, De Becker, Ann, Schots, Rik
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7692351/
https://www.ncbi.nlm.nih.gov/pubmed/33281955
http://dx.doi.org/10.1177/1759720X20974858
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author Ajeganova, Sofia
De Becker, Ann
Schots, Rik
author_facet Ajeganova, Sofia
De Becker, Ann
Schots, Rik
author_sort Ajeganova, Sofia
collection PubMed
description Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.
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spelling pubmed-76923512020-12-04 Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance Ajeganova, Sofia De Becker, Ann Schots, Rik Ther Adv Musculoskelet Dis Case Report Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission. SAGE Publications 2020-11-24 /pmc/articles/PMC7692351/ /pubmed/33281955 http://dx.doi.org/10.1177/1759720X20974858 Text en © The Author(s), 2020 https://creativecommons.org/licenses/by/4.0/ This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Ajeganova, Sofia
De Becker, Ann
Schots, Rik
Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title_full Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title_fullStr Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title_full_unstemmed Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title_short Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance
title_sort efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset still’s disease: when dosage matters in overcoming secondary therapy resistance
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7692351/
https://www.ncbi.nlm.nih.gov/pubmed/33281955
http://dx.doi.org/10.1177/1759720X20974858
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