IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature

Immunoglobin-G4 related disease (IgG4-RD) is an auto-immune inflammatory condition where patients present with a tumour-like mass that shows infiltration by plasma cell and subsequent fibrosis. It is a systemic condition that primarily involves the salivary glands, pancreas, kidneys, aorta, and retr...

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Main Authors: Asbah SHAMS, Abhijit DAS, Madhu SINHA, Natasha GULATI, Man Mohan MEHNDIRATTA, Manish KAUSHIK, Puneet GUPTA
Format: Article
Language:English
Published: Federation of Turkish Pathology Societies 2021-01-01
Series:Türk Patoloji Dergisi
Subjects:
Online Access: http://www.turkjpath.org/pdf.php3?id=1928
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author Asbah SHAMS
Abhijit DAS
Madhu SINHA
Natasha GULATI
Man Mohan MEHNDIRATTA
Manish KAUSHIK
Puneet GUPTA
author_facet Asbah SHAMS
Abhijit DAS
Madhu SINHA
Natasha GULATI
Man Mohan MEHNDIRATTA
Manish KAUSHIK
Puneet GUPTA
author_sort Asbah SHAMS
collection DOAJ
description Immunoglobin-G4 related disease (IgG4-RD) is an auto-immune inflammatory condition where patients present with a tumour-like mass that shows infiltration by plasma cell and subsequent fibrosis. It is a systemic condition that primarily involves the salivary glands, pancreas, kidneys, aorta, and retroperitoneum amongst other organs. Testicular involvement is a rare occurrence in this disease entity. A 55-year old male patient presented with the complaints of pain and swelling in the right scrotal region. Right-sided orchidectomy was carried out which on histopathology showed features suggestive of IgG4-RD which was later confirmed on immunohistochemistry. Whole body MRI revealed that no other organ was involved in the disease process in this patient. IgG4-RD has a variable clinical course and considerable overlap with its differentials. Imaging studies and serum IgG4 levels are neither confirmatory nor customarily diagnostic in every case. The only confirmatory diagnostic investigation is histopathological examination, which shows infiltration of IgG4+ plasma cells and fibrosis in the involved tissue. Whenever a mass-forming lesion with typical histomorphological features is encountered with involvement of multiple organs/anatomic sites, IgG4-related disease should be considered among the differentials, and clinicians of all disciplines should be familiar with this disease entity.
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spelling doaj.art-4226cb7b6f4745abb9fbbbc87e98452d2023-02-15T16:21:16ZengFederation of Turkish Pathology SocietiesTürk Patoloji Dergisi1018-56151309-57302021-01-0137107808310.5146/tjpath.2020.01493IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of LiteratureAsbah SHAMSAbhijit DASMadhu SINHANatasha GULATIMan Mohan MEHNDIRATTAManish KAUSHIKPuneet GUPTAImmunoglobin-G4 related disease (IgG4-RD) is an auto-immune inflammatory condition where patients present with a tumour-like mass that shows infiltration by plasma cell and subsequent fibrosis. It is a systemic condition that primarily involves the salivary glands, pancreas, kidneys, aorta, and retroperitoneum amongst other organs. Testicular involvement is a rare occurrence in this disease entity. A 55-year old male patient presented with the complaints of pain and swelling in the right scrotal region. Right-sided orchidectomy was carried out which on histopathology showed features suggestive of IgG4-RD which was later confirmed on immunohistochemistry. Whole body MRI revealed that no other organ was involved in the disease process in this patient. IgG4-RD has a variable clinical course and considerable overlap with its differentials. Imaging studies and serum IgG4 levels are neither confirmatory nor customarily diagnostic in every case. The only confirmatory diagnostic investigation is histopathological examination, which shows infiltration of IgG4+ plasma cells and fibrosis in the involved tissue. Whenever a mass-forming lesion with typical histomorphological features is encountered with involvement of multiple organs/anatomic sites, IgG4-related disease should be considered among the differentials, and clinicians of all disciplines should be familiar with this disease entity. http://www.turkjpath.org/pdf.php3?id=1928 igg4-related diseasetestisplasma cellsfibrosis
spellingShingle Asbah SHAMS
Abhijit DAS
Madhu SINHA
Natasha GULATI
Man Mohan MEHNDIRATTA
Manish KAUSHIK
Puneet GUPTA
IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
Türk Patoloji Dergisi
igg4-related disease
testis
plasma cells
fibrosis
title IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
title_full IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
title_fullStr IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
title_full_unstemmed IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
title_short IgG4-Related Disease with Selective Testicular Involvement- A Rare Entity: Case Report with Review of Literature
title_sort igg4 related disease with selective testicular involvement a rare entity case report with review of literature
topic igg4-related disease
testis
plasma cells
fibrosis
url http://www.turkjpath.org/pdf.php3?id=1928
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