A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that typically manifests as mass lesions affecting almost any organ including the pancreas, lacrimal and salivary glands, liver, lung and kidney. However, IgG4-RD with urethra involvement is scarce. We descri...

Full description

Bibliographic Details
Main Authors: Zhiru Zeng, Shasha Gao, Xiaoyong Lu
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-01-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2022.1077609/full
_version_ 1797939109057003520
author Zhiru Zeng
Shasha Gao
Xiaoyong Lu
author_facet Zhiru Zeng
Shasha Gao
Xiaoyong Lu
author_sort Zhiru Zeng
collection DOAJ
description Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that typically manifests as mass lesions affecting almost any organ including the pancreas, lacrimal and salivary glands, liver, lung and kidney. However, IgG4-RD with urethra involvement is scarce. We describe a rare case of IgG4-RD involving the urethra mimicking urethral carcinoma and review the published literature. A 64-years-old female presented with progressive dysuria for more than 2 months. Pelvic gadolinium-enhanced magnetic resonance imaging revealed a huge mass encasing the urethra which showed obvious enhancement in the arterial phase. And contrast-enhanced ultrasound showed that the entire mass was heterogeneously enhanced and displayed a fast-forward and fast-out pattern, which was highly suggestive of malignant tumor. The diagnosis of IgG4-RD was finally established by ultrasound-guided transvaginal mass needle biopsy. The patient was treated with methylprednisolone and cyclophosphamide and dysuria disappeared in the first week of therapy. She has been followed up in our clinic for 1 year without recurrence. The diagnosis of IgG4-RD should be considered in the differential diagnosis of a periurethral mass. Ultrasound-guided transvaginal mass needle biopsy is a safe and well-established tissue sampling method and should be performed in order to avoid unnecessary surgery.
first_indexed 2024-04-10T19:11:13Z
format Article
id doaj.art-868738aa9d7b4d5db421ed425780f6c5
institution Directory Open Access Journal
issn 1664-3224
language English
last_indexed 2024-04-10T19:11:13Z
publishDate 2023-01-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Immunology
spelling doaj.art-868738aa9d7b4d5db421ed425780f6c52023-01-30T17:18:38ZengFrontiers Media S.A.Frontiers in Immunology1664-32242023-01-011310.3389/fimmu.2022.10776091077609A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literatureZhiru Zeng0Shasha Gao1Xiaoyong Lu2Department of Rheumatology, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, ChinaDepartment of Endocrinology and Rheumatology, Anji Traditional Chinese Medical Hospital, Huzhou, Zhejiang, ChinaDepartment of Rheumatology, the Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, ChinaImmunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disease that typically manifests as mass lesions affecting almost any organ including the pancreas, lacrimal and salivary glands, liver, lung and kidney. However, IgG4-RD with urethra involvement is scarce. We describe a rare case of IgG4-RD involving the urethra mimicking urethral carcinoma and review the published literature. A 64-years-old female presented with progressive dysuria for more than 2 months. Pelvic gadolinium-enhanced magnetic resonance imaging revealed a huge mass encasing the urethra which showed obvious enhancement in the arterial phase. And contrast-enhanced ultrasound showed that the entire mass was heterogeneously enhanced and displayed a fast-forward and fast-out pattern, which was highly suggestive of malignant tumor. The diagnosis of IgG4-RD was finally established by ultrasound-guided transvaginal mass needle biopsy. The patient was treated with methylprednisolone and cyclophosphamide and dysuria disappeared in the first week of therapy. She has been followed up in our clinic for 1 year without recurrence. The diagnosis of IgG4-RD should be considered in the differential diagnosis of a periurethral mass. Ultrasound-guided transvaginal mass needle biopsy is a safe and well-established tissue sampling method and should be performed in order to avoid unnecessary surgery.https://www.frontiersin.org/articles/10.3389/fimmu.2022.1077609/fullimmunoglobulin G4-related diseaseurethraperiurethral massdysuriacontrast-enhanced ultrasound (CEUS)
spellingShingle Zhiru Zeng
Shasha Gao
Xiaoyong Lu
A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
Frontiers in Immunology
immunoglobulin G4-related disease
urethra
periurethral mass
dysuria
contrast-enhanced ultrasound (CEUS)
title A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
title_full A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
title_fullStr A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
title_full_unstemmed A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
title_short A rare case of IgG4-related disease masquerading as periurethral malignancy and review of the literature
title_sort rare case of igg4 related disease masquerading as periurethral malignancy and review of the literature
topic immunoglobulin G4-related disease
urethra
periurethral mass
dysuria
contrast-enhanced ultrasound (CEUS)
url https://www.frontiersin.org/articles/10.3389/fimmu.2022.1077609/full
work_keys_str_mv AT zhiruzeng ararecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature
AT shashagao ararecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature
AT xiaoyonglu ararecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature
AT zhiruzeng rarecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature
AT shashagao rarecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature
AT xiaoyonglu rarecaseofigg4relateddiseasemasqueradingasperiurethralmalignancyandreviewoftheliterature