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Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review

Introduction: Biological disease-modifying anti-rheumatic drugs (bDMARDs) targeting interleukin (IL)-6 and IL-1β represent a steroid-sparing first-line therapy used in systemic-onset juvenile idiopathic arthritis (sJIA). Recently, the occurrence of pulmonary alveolar proteinosis (PAP) in sJIA patien...

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Autores principales: Zekre, Franck, Duncan, Anita, Laurent, Audrey, Tusseau, Maud, Pescarmona, Rémi, Collardeau-Frachon, Sophie, Ohlmann, Camille, Viel, Sébastien, Reix, Philippe, Benezech, Sarah, Belot, Alexandre
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10095381/
https://www.ncbi.nlm.nih.gov/pubmed/37048785
http://dx.doi.org/10.3390/jcm12072702
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author Zekre, Franck
Duncan, Anita
Laurent, Audrey
Tusseau, Maud
Pescarmona, Rémi
Collardeau-Frachon, Sophie
Ohlmann, Camille
Viel, Sébastien
Reix, Philippe
Benezech, Sarah
Belot, Alexandre
author_facet Zekre, Franck
Duncan, Anita
Laurent, Audrey
Tusseau, Maud
Pescarmona, Rémi
Collardeau-Frachon, Sophie
Ohlmann, Camille
Viel, Sébastien
Reix, Philippe
Benezech, Sarah
Belot, Alexandre
author_sort Zekre, Franck
collection PubMed
description Introduction: Biological disease-modifying anti-rheumatic drugs (bDMARDs) targeting interleukin (IL)-6 and IL-1β represent a steroid-sparing first-line therapy used in systemic-onset juvenile idiopathic arthritis (sJIA). Recently, the occurrence of pulmonary alveolar proteinosis (PAP) in sJIA patients was reported with early-onset and exposure to bDMARDs as potential risk factors. We report on a new case with longitudinal immunomonitoring successfully treated by Janus Kinase inhibitors (JAKi) and review past clinical descriptions of this new entity. Methods: We report one case of pulmonary alveolar proteinosis and macrophage activation syndrome (PAP-MAS) with longitudinal immunomonitoring. We then conducted a review of the literature of seven publications reporting 107 cases of PAP-MAS sJIA, and included the main characteristics and evolution under treatment. Results: Of the seven articles analyzed, the incidence of PAP-MAS among sJIA patients varied from 1.28% to 12.9%. We report here a single case among a cohort of 537 sJIA patients followed in the pediatric department of the Hospices Civils de Lyon over the last 15 years. This child presented with all clinical and immunological characteristics of PAP-MAS. After several lines of treatment, he benefited from JAKi and improved with respect to both systemic symptoms and lung disease. In the literature, strategies with monoclonal antibodies targeting either INF-γ or IL-1β/IL-18 have been tested with variable results. Orally taken JAKi presents the advantage of targeting multiple cytokines and avoiding parenteral injections of monoclonal antibodies that may contribute to the pathogenesis. Conclusions: JAKi represent a promising option in the treatment of lung disease associated with sJIA.
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spelling pubmed-100953812023-04-13 Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review Zekre, Franck Duncan, Anita Laurent, Audrey Tusseau, Maud Pescarmona, Rémi Collardeau-Frachon, Sophie Ohlmann, Camille Viel, Sébastien Reix, Philippe Benezech, Sarah Belot, Alexandre J Clin Med Brief Report Introduction: Biological disease-modifying anti-rheumatic drugs (bDMARDs) targeting interleukin (IL)-6 and IL-1β represent a steroid-sparing first-line therapy used in systemic-onset juvenile idiopathic arthritis (sJIA). Recently, the occurrence of pulmonary alveolar proteinosis (PAP) in sJIA patients was reported with early-onset and exposure to bDMARDs as potential risk factors. We report on a new case with longitudinal immunomonitoring successfully treated by Janus Kinase inhibitors (JAKi) and review past clinical descriptions of this new entity. Methods: We report one case of pulmonary alveolar proteinosis and macrophage activation syndrome (PAP-MAS) with longitudinal immunomonitoring. We then conducted a review of the literature of seven publications reporting 107 cases of PAP-MAS sJIA, and included the main characteristics and evolution under treatment. Results: Of the seven articles analyzed, the incidence of PAP-MAS among sJIA patients varied from 1.28% to 12.9%. We report here a single case among a cohort of 537 sJIA patients followed in the pediatric department of the Hospices Civils de Lyon over the last 15 years. This child presented with all clinical and immunological characteristics of PAP-MAS. After several lines of treatment, he benefited from JAKi and improved with respect to both systemic symptoms and lung disease. In the literature, strategies with monoclonal antibodies targeting either INF-γ or IL-1β/IL-18 have been tested with variable results. Orally taken JAKi presents the advantage of targeting multiple cytokines and avoiding parenteral injections of monoclonal antibodies that may contribute to the pathogenesis. Conclusions: JAKi represent a promising option in the treatment of lung disease associated with sJIA. MDPI 2023-04-04 /pmc/articles/PMC10095381/ /pubmed/37048785 http://dx.doi.org/10.3390/jcm12072702 Text en © 2023 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 Brief Report
Zekre, Franck
Duncan, Anita
Laurent, Audrey
Tusseau, Maud
Pescarmona, Rémi
Collardeau-Frachon, Sophie
Ohlmann, Camille
Viel, Sébastien
Reix, Philippe
Benezech, Sarah
Belot, Alexandre
Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title_full Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title_fullStr Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title_full_unstemmed Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title_short Rescue of Pap-Mas in Systemic JIA Using Janus Kinase Inhibitors, Case Report and Systematic Review
title_sort rescue of pap-mas in systemic jia using janus kinase inhibitors, case report and systematic review
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10095381/
https://www.ncbi.nlm.nih.gov/pubmed/37048785
http://dx.doi.org/10.3390/jcm12072702
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