AUTHOR=Li Na , Xu LiFen , Liu Xiaoxia , Wu Pengjia , Liu Jun , Zeng Jiashun 

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

JOURNAL=Frontiers in Immunology

VOLUME=Volume 16 - 2025

YEAR=2025

URL=https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2025.1532627

DOI=10.3389/fimmu.2025.1532627

ISSN=1664-3224

ABSTRACT=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.