Title:Immunoglobulin G4-related Disease with Multiple Organs Involvement
Depicted on FDG PET/CT: A Case Report and Literature Review
Volume: 20
Author(s): Xiaoying Zhu, Lili Wu, Panpan Lv, Yongmei han and Yiyu Zhuang*
Affiliation:
- Department of Nursing, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, P.R. China
Keywords:
IgG4-related disease, Submandibular mass, Olfactory dysfunction, PET/CT, Monoclonal immunoglobulin, Anosmia.
Abstract:
Introduction:
Immunoglobulin G4-related disease (IgG4-RD) is a relatively rare immune-mediated chronic inflammatory disease with fibrosis newly defined in
recent years. It can involve multiple systems and organs with complex clinical manifestations. Due to mass-like lesions, it is easily misdiagnosed as
tumors.
Case Report:
Herein, we report a 57-year-old woman treated for submandibular mass and anosmia. The serum IgG4 level was increased. The biopsy of the
submandibular gland indicated salivary gland tissue and hyperplasia of fibrous tissue and lymphoid tissue. Immunohistochemical examination
showed a large number of IgG4-positive plasma cells. M protein was found in the patient's serum by immunofixation electrophoresis, and plasma
cell diseases were excluded by bone marrow puncture. PET/CT examination showed that besides the submandibular glands, the parotid gland,
common bile duct, the transitional part of the left renal pelvis and ureter, retroperitoneum in the lower abdomen, and multiple lymph nodes were
also involved. The patient was diagnosed with IgG4-RD, and after treatment with glucocorticoid, the enlargement of submandibular glands and
decreased olfactory function improved. After 14 weeks of treatment, the serological examinations, PET/CT, and ultrasound re-examination results
showed significant improvement. So far, the patient has been followed up for 27 months and is in continuous remission.
Conclusion:
This case report aims to raise awareness of IgG4-RD and explore the value of PET/CT in the diagnosis and efficacy monitoring of the disease.
