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...

Full description

Saved in:
Bibliographic Details
Published in:Frontiers in immunology 2019-08, Vol.10, p.1910
Main Authors: van den Elshout-den Uyl, Debby, Spoto, Clothaire P E, de Boer, Mirthe, Leiner, Tim, Leavis, Helen L, Leguit, Roos J
Format: Article
Language:English
Subjects:
bone marrow
IgG4-related disease
Immunology
MRI
plasma cell
spine
vertebra
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1664-3224
1664-3224
DOI:10.3389/fimmu.2019.01910