Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report

Castleman disease (CD) and immunoglobulin G4-related disease (IgG4-RD) are rare systemic immune-mediated disorders that share overlapping clinical features, posing significant challenges in differential diagnosis. Here, we present a case of generalized lymphadenopathy initially misdiagnosed as IgG4-...

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Main Authors: Na Li, LiFen Xu, Xiaoxia Liu, Pengjia Wu, Jun Liu, Jiashun Zeng
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-01-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fimmu.2025.1532627/full
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author Na Li
LiFen Xu
Xiaoxia Liu
Pengjia Wu
Jun Liu
Jiashun Zeng
author_facet Na Li
LiFen Xu
Xiaoxia Liu
Pengjia Wu
Jun Liu
Jiashun Zeng
author_sort Na Li
collection DOAJ
description Castleman disease (CD) and immunoglobulin G4-related disease (IgG4-RD) are rare systemic immune-mediated disorders that share overlapping clinical features, posing significant challenges in differential diagnosis. Here, we present a case of generalized lymphadenopathy initially misdiagnosed as IgG4-RD, which demonstrated a poor response to hormonal therapy. Subsequent pathological biopsy and immunohistochemical analysis ultimately confirmed the diagnosis of CD. This case underscores the limitations of relying solely on serum IgG4 levels as a diagnostic marker to distinguish CD from IgG4-RD. Comprehensive evaluation, including clinical presentation, organ involvement, serological and pathological findings, as well as therapeutic response, is essential to ensure accurate diagnosis and timely management.
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institution Kabale University
issn 1664-3224
language English
publishDate 2025-01-01
publisher Frontiers Media S.A.
record_format Article
series Frontiers in Immunology
spelling doaj-art-73d6feef18da48fdbc7653725343e9502025-01-28T06:41:20ZengFrontiers Media S.A.Frontiers in Immunology1664-32242025-01-011610.3389/fimmu.2025.15326271532627Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case reportNa Li0LiFen Xu1Xiaoxia Liu2Pengjia Wu3Jun Liu4Jiashun Zeng5Department of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaPathology Department, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaDepartment of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaDepartment of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaDepartment of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaDepartment of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, ChinaCastleman disease (CD) and immunoglobulin G4-related disease (IgG4-RD) are rare systemic immune-mediated disorders that share overlapping clinical features, posing significant challenges in differential diagnosis. Here, we present a case of generalized lymphadenopathy initially misdiagnosed as IgG4-RD, which demonstrated a poor response to hormonal therapy. Subsequent pathological biopsy and immunohistochemical analysis ultimately confirmed the diagnosis of CD. This case underscores the limitations of relying solely on serum IgG4 levels as a diagnostic marker to distinguish CD from IgG4-RD. Comprehensive evaluation, including clinical presentation, organ involvement, serological and pathological findings, as well as therapeutic response, is essential to ensure accurate diagnosis and timely management.https://www.frontiersin.org/articles/10.3389/fimmu.2025.1532627/fullCastleman diseaseIgG4-related diseaseimmune-mediated disorderslymphadenopathyaccurate diagnosis
spellingShingle Na Li
LiFen Xu
Xiaoxia Liu
Pengjia Wu
Jun Liu
Jiashun Zeng
Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
Frontiers in Immunology
Castleman disease
IgG4-related disease
immune-mediated disorders
lymphadenopathy
accurate diagnosis
title Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
title_full Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
title_fullStr Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
title_full_unstemmed Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
title_short Castleman disease misdiagnosed as immunoglobulin G4-related disease: a case report
title_sort castleman disease misdiagnosed as immunoglobulin g4 related disease a case report
topic Castleman disease
IgG4-related disease
immune-mediated disorders
lymphadenopathy
accurate diagnosis
url https://www.frontiersin.org/articles/10.3389/fimmu.2025.1532627/full
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AT pengjiawu castlemandiseasemisdiagnosedasimmunoglobuling4relateddiseaseacasereport
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