Paraneoplastic Chronic Periaortitis with Histologic Findings Mimicking IgG4-related Disease

Abstract

Chronic periaortitis is usually idiopathic but may occur secondary to malignancy or IgG4-related disease (IgG4-RD). We report a case of a 56-year-old man with dysphonia caused by recurrent laryngeal nerve compression due to periaortic soft tissue thickening. A histological examination revealed storiform fibrosis and lymphoplasmacytic infiltration, but not elevated serum IgG4 levels or abundant IgG4-positive cells. Imaging revealed progressive involvement of the left common carotid artery, and FDG-PET showed a focal uptake. Metastatic papillary thyroid carcinoma was subsequently identified in the adjacent lymph nodes. Periaortitis improved with glucocorticoids after surgery. This case highlights paraneoplastic periaortitis mimicking IgG4-RD and the need to screen for malignancy when the findings are inconclusive.

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Cite

BibTeX RIS

BibTeX

@article{goto2026paraneoplastic,
  title = {{Paraneoplastic Chronic Periaortitis with Histologic Findings Mimicking IgG4-related Disease}},
  author = {Ayane Goto and Toshihiko Komai and Shinji Izuka and Toshiaki Shimizu and Mariko Tanaka and Hiroaki Harada and Hirofumi Shoda and Keishi Fujio},
  journal = {Internal Medicine},
  year = {2026},
  month = sep,
  doi = {10.2169/internalmedicine.7078-26},
  url = {https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7078-26/_article},
  note = {CC BY-NC-ND},
}

RIS

TY  - JOUR
TI  - Paraneoplastic Chronic Periaortitis with Histologic Findings Mimicking IgG4-related Disease
AU  - Ayane Goto
AU  - Toshihiko Komai
AU  - Shinji Izuka
AU  - Toshiaki Shimizu
AU  - Mariko Tanaka
AU  - Hiroaki Harada
AU  - Hirofumi Shoda
AU  - Keishi Fujio
PY  - 2026
DA  - 2026/09/15
JO  - Internal Medicine
DO  - 10.2169/internalmedicine.7078-26
UR  - https://www.jstage.jst.go.jp/article/internalmedicine/advpub/0/advpub_7078-26/_article
AB  - Chronic periaortitis is usually idiopathic but may occur secondary to malignancy or IgG4-related disease (IgG4-RD). We report a case of a 56-year-old man with dysphonia caused by recurrent laryngeal nerve compression due to periaortic soft tissue thickening. A histological examination revealed storiform fibrosis and lymphoplasmacytic infiltration, but not elevated serum IgG4 levels or abundant IgG4-positive cells. Imaging revealed progressive involvement of the left common carotid artery, and FDG-PET showed a focal uptake. Metastatic papillary thyroid carcinoma was subsequently identified in the adjacent lymph nodes. Periaortitis improved with glucocorticoids after surgery. This case highlights paraneoplastic periaortitis mimicking IgG4-RD and the need to screen for malignancy when the findings are inconclusive.
SN  - 1349-7235
SN  - 0918-2918
ER  -