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

Publication date

2019-01-01

Authors

van den Elshout-Den Uyl, Debby
Spoto, Clothaire
de Boer, M. G. J.
Leiner, TimORCID 0000-0003-1885-5499ISNI 0000000390698205
Leavis, HelenISNI 0000000391805033
Leguit, Roos JISNI 0000000393502220

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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.

Keywords

Bone marrow, Histology, IgG4-related disease, Immunhistochemistry, MRI, Plasma cell, Spine, Vertebra, Immunology and Allergy, Immunology, Case Reports

Citation

van den Elshout-Den Uyl, D, Spoto, C P E, de Boer, M, Leiner, T, Leavis, H L & Leguit, R J 2019, 'First report of IgG4 related disease primary presenting as vertebral bone marrow lesions', Frontiers in Immunology, vol. 10, no. AUG, 1910. https://doi.org/10.3389/fimmu.2019.01910