IgG4‐Related Disease Inflamatory Pseudotumor Affecting the Sternoclavicular Joint: A Clinical Challenge

IgG4-related disease (IgG4-RD) is a rare, progressive, and immune-mediated fibroinflammatory disorder that primarily affects middle-aged men and is more prevalent in Asian populations. Although extensively studied, its pathophysiology remains incompletely understood. This case report describes a 44-year-old male presenting with multiple abscesses and progressive inflammatory symptoms, ultimately diagnosed with IgG4-RD with musculoskeletal involvement. Imaging and histopathological evaluation confirmed osteolytic lesions and significant IgG4-positive plasma cell infiltration. Soft tissue tumors in IgG4-RD are exceptionally rare, further emphasizing the uniqueness of this case. The patient showed clinical improvement with corticosteroid therapy. This case highlights the importance of considering IgG4-RD in the differential diagnosis of soft tissue and bone lesions and underscores the need for a multidisciplinary diagnostic approach.

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Publication Details

Journal
Case Reports in Immunology
Published
2025-01-01
DOI
https://doi.org/10.1155/crii/6543528
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
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article

IgG4‐Related Disease Inflamatory Pseudotumor Affecting the Sternoclavicular Joint: A Clinical Challenge

Andre Luis Coutinho de Araújo Macedo, João Paulo Uchoa Fontenele, Talita Guimarães Andrade, Orivaldo Alves Barbosa et al.
Case Reports in Immunology
IgG4-Related and Inflammatory Diseases
article

IgG4‐Related Disease Inflamatory Pseudotumor Affecting the Sternoclavicular Joint: A Clinical Challenge

Andre Luis Coutinho de Araújo Macedo, João Paulo Uchoa Fontenele, Talita Guimarães Andrade, Orivaldo Alves Barbosa, José Walter Correia, Dower Frota Barroso, Sergio Saldanha Freire Simões
article en

Abstract

IgG4-related disease (IgG4-RD) is a rare, progressive, and immune-mediated fibroinflammatory disorder that primarily affects middle-aged men and is more prevalent in Asian populations. Although extensively studied, its pathophysiology remains incompletely understood. This case report describes a 44-year-old male presenting with multiple abscesses and progressive inflammatory symptoms, ultimately diagnosed with IgG4-RD with musculoskeletal involvement. Imaging and histopathological evaluation confirmed osteolytic lesions and significant IgG4-positive plasma cell infiltration. Soft tissue tumors in IgG4-RD are exceptionally rare, further emphasizing the uniqueness of this case. The patient showed clinical improvement with corticosteroid therapy. This case highlights the importance of considering IgG4-RD in the differential diagnosis of soft tissue and bone lesions and underscores the need for a multidisciplinary diagnostic approach.

Case Reports in ImmunologyVol. 2025(1)
Hospital Infantil Albert Sabin (BR), Hospital Geral de Fortaleza (BR)
Openalex Percentile: Top 66%
IgG4-Related and Inflammatory Diseases
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IgG4‐Related Disease Inflamatory Pseudotumor Affecting the Sternoclavicular Joint: A Clinical Challenge — Andre Luis Coutinho de Araújo Macedo, João Paulo Uchoa Fontenele, et al. · Case Reports in Immunology (2025) | TGRS Research Map | TGRS