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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Main Authors:	Debby van den Elshout-den Uyl, Clothaire P. E. Spoto, Mirthe de Boer, Tim Leiner, Helen L. Leavis, Roos J. Leguit
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2019-08-01
Series:	Frontiers in Immunology
Subjects:	IgG4-related disease spine MRI bone marrow vertebra plasma cell
Online Access:	https://www.frontiersin.org/article/10.3389/fimmu.2019.01910/full

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spelling	doaj-3525c229ec044dac8f62dc166067bee12020-11-24T22:14:36ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-08-011010.3389/fimmu.2019.01910454887First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow LesionsDebby van den Elshout-den Uyl0Clothaire P. E. Spoto1Mirthe de Boer2Tim Leiner3Helen L. Leavis4Roos J. Leguit5Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Pathology, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Pathology, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Radiology, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, NetherlandsDepartment of Pathology, University Medical Center Utrecht, Utrecht, NetherlandsIgG4-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.https://www.frontiersin.org/article/10.3389/fimmu.2019.01910/fullIgG4-related diseasespineMRIbone marrowvertebraplasma cell
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	Debby van den Elshout-den Uyl Clothaire P. E. Spoto Mirthe de Boer Tim Leiner Helen L. Leavis Roos J. Leguit
spellingShingle	Debby van den Elshout-den Uyl Clothaire P. E. Spoto Mirthe de Boer Tim Leiner Helen L. Leavis Roos J. Leguit First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions Frontiers in Immunology IgG4-related disease spine MRI bone marrow vertebra plasma cell
author_facet	Debby van den Elshout-den Uyl Clothaire P. E. Spoto Mirthe de Boer Tim Leiner Helen L. Leavis Roos J. Leguit
author_sort	Debby van den Elshout-den Uyl
title	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_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_sort	first report of igg4 related disease primary presenting as vertebral bone marrow lesions
publisher	Frontiers Media S.A.
series	Frontiers in Immunology
issn	1664-3224
publishDate	2019-08-01
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.
topic	IgG4-related disease spine MRI bone marrow vertebra plasma cell
url	https://www.frontiersin.org/article/10.3389/fimmu.2019.01910/full
work_keys_str_mv	AT debbyvandenelshoutdenuyl firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions AT clothairepespoto firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions AT mirthedeboer firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions AT timleiner firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions AT helenlleavis firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions AT roosjleguit firstreportofigg4relateddiseaseprimarypresentingasvertebralbonemarrowlesions
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First Report of IgG4 Related Disease Primary Presenting as Vertebral Bone Marrow Lesions

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