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Paraneoplastic Chronic Periaortitis with Histologic Findings Mimicking IgG4-related Disease
Ayane Goto1, Toshihiko Komai1, Shinji Izuka1
1Department of Allergy and Rheumatology, Graduate School of Medicine, The University of Tokyo, Japan.
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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