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Spinal Bony Involvement of IgG4-related Disease Treated by a Spondylectomy

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Abstract
Immunoglobulin G4-related sclerosing disease (IgG4RD) is an emerging immune-mediated fibro-inflammatory
disorder which can involve any organ. We describe the first IgG4-RD spondylitis treated
with total en-bloc spondylectomy (TES). A 55-year-old man presented with back pain. Magnetic
resonance imaging (MRI) of the thoracic spine revealed a pathologic compression fracture on T11
vertebral body and both pedicles suggestive of primary bone tumor or bone metastasis. We conducted
TES of T11, because we could not exclude the possibility of primary bone tumor including
giant cell tumor. Immunohistochemical examination of the pathology specimens from pleura around
the pedicle demonstrated diffuse infiltration of IgG4-bearing plasma cells. Six weeks later from the
surgery, a delayed serologic test was done and his serum IgG4 concentration was 45 mg/dL. The final
diagnosis was probable IgG4RD on the basis of serological, imaging, histopathological findings.
After 6 weeks of oral prednisolone treatment, patient’s back pain improved dramatically. IgG4RD is
very rare systemic disease and its paraspinal soft tissue like pleura involvement with vertebra body
invasion was absent until now. Our experience indicated that surrounding soft tissue biopsy would
be helpful when a percutaneous vertebra bone biopsy mismatched with the image studies, even
though vertebra body was main pathological lesion considering the possibility of IgG4RD.
Author(s)
김동진이수범정희정홍석찬김민재정상구박진훈
Issued Date
2021
Type
Article
Keyword
immunoglobulin G4-related diseasespinal bony involvement
DOI
10.2176/nmccrj.cr.2020-0067
URI
https://oak.ulsan.ac.kr/handle/2021.oak/7629
https://ulsan-primo.hosted.exlibrisgroup.com/primo-explore/fulldisplay?docid=TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_8116914&context=PC&vid=ULSAN&lang=ko_KR&search_scope=default_scope&adaptor=primo_central_multiple_fe&tab=default_tab&query=any,contains,Spinal%20Bony%20Involvement%20of%20IgG4-related%20Disease%20Treated%20by%20a%20Spondylectomy&offset=0&pcAvailability=true
Publisher
NMC Case Report Journal
Location
일본
Language
영어
ISSN
2188-4226
Citation Volume
8
Citation Number
1
Citation Start Page
27
Citation End Page
31
Appears in Collections:
Medicine > Medicine
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