First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions

IgG4-related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, including, but rarely, bone marrowinvolvement. Her...

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Autores principales: van den Elshout-den Uyl, Debby, Spoto, Clothaire P. E., de Boer, Mirthe, Leiner, Tim, Leavis, Helen L., Leguit, Roos J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Immunology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6700296/
https://www.ncbi.nlm.nih.gov/pubmed/31456806
http://dx.doi.org/10.3389/fimmu.2019.01910
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author van den Elshout-den Uyl, Debby
Spoto, Clothaire P. E.
de Boer, Mirthe
Leiner, Tim
Leavis, Helen L.
Leguit, Roos J.
author_facet van den Elshout-den Uyl, Debby
Spoto, Clothaire P. E.
de Boer, Mirthe
Leiner, Tim
Leavis, Helen L.
Leguit, Roos J.
author_sort van den Elshout-den Uyl, Debby
collection PubMed
description IgG4-related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, including, but rarely, bone marrowinvolvement. Here we present a case of a 76-year-old male with IgG4-related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storiform fibrosis, and increased IgG4 positive plasma cells. Treatment with corticosteroids significantly improved wellbeing and resolved lesion size on MRI.
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spelling pubmed-67002962019-08-27 First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions van den Elshout-den Uyl, Debby Spoto, Clothaire P. E. de Boer, Mirthe Leiner, Tim Leavis, Helen L. Leguit, Roos J. Front Immunol Immunology IgG4-related disease is a fibro-inflammatory disorder characterized by swelling of tissues and affected organs accompanied by the development of scar tissue (fibrosis) and infiltration by IgG4 positive plasma cells. Almost any organ can be affected, including, but rarely, bone marrowinvolvement. Here we present a case of a 76-year-old male with IgG4-related disease presenting primarily with vertebral bone marrow lesions. Histopathology showed the typical features of storiform fibrosis, and increased IgG4 positive plasma cells. Treatment with corticosteroids significantly improved wellbeing and resolved lesion size on MRI. Frontiers Media S.A. 2019-08-13 /pmc/articles/PMC6700296/ /pubmed/31456806 http://dx.doi.org/10.3389/fimmu.2019.01910 Text en Copyright © 2019 van den Elshout-den Uyl, Spoto, de Boer, Leiner, Leavis and Leguit. http://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 Immunology
van den Elshout-den Uyl, Debby
Spoto, Clothaire P. E.
de Boer, Mirthe
Leiner, Tim
Leavis, Helen L.
Leguit, Roos J.
First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title_full First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title_fullStr First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title_full_unstemmed First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title_short First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions
title_sort first report of igg4 related disease primary presenting as vertebral bone marrow lesions
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6700296/
https://www.ncbi.nlm.nih.gov/pubmed/31456806
http://dx.doi.org/10.3389/fimmu.2019.01910
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