First report of IgG4 related disease primary presenting as vertebral bone marrow lesions

Debby van den Elshout-Den Uyl; Clothaire P.E. Spoto; Mirthe de Boer; Tim Leiner; Helen L. Leavis; Roos J. Leguit

doi:10.3389/fimmu.2019.01910

First report of IgG4 related disease primary presenting as vertebral bone marrow lesions

Debby van den Elshout-Den Uyl^*, Clothaire P.E. Spoto, Mirthe de Boer, Tim Leiner, Helen L. Leavis, Roos J. Leguit

^*Corresponding author for this work

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Abstract

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.

Original language	English
Article number	1910
Number of pages	5
Journal	Frontiers in Immunology
Volume	10
Issue number	AUG
DOIs	https://doi.org/10.3389/fimmu.2019.01910
Publication status	Published - 1 Jan 2019

Keywords

Bone marrow
Histology
IgG4-related disease
Immunhistochemistry
MRI
Plasma cell
Spine
Vertebra

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title = "First report of IgG4 related disease primary presenting as vertebral bone marrow lesions",

abstract = "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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AU - de Boer, Mirthe

AU - Leiner, Tim

AU - Leavis, Helen L.

AU - Leguit, Roos J.

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AB - 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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KW - Histology

KW - IgG4-related disease

KW - Immunhistochemistry

KW - MRI

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KW - Spine

KW - Vertebra

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First report of IgG4 related disease primary presenting as vertebral bone marrow lesions

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